| PC0121: Biological usage in spondyloarthritis: A single centre observation |  |
Nagaprabu V N, Velammal P1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India
Introduction: Spondyloarthritis(SPA) is a group of disease with limited therapeutic options unlike rheumatoid arthritis. The usage of Biologicals have increased considerably to achieve disease control.
Materials and Methods: We analyzed the outcome of patients who received Biologicals in our centre during June 2014 - July 2019. Patients with less than 6 months post biological follow-up were excluded. The patients had received Biologicals as per the disease activity assessment and not as per the schedule.
Observation: There were a total of 71 patients who were included for the analysis of which there were 52 males and 19 females. The mean age of the study population was 37.43 ± 13.01 years. In the study population there were 11 psoriatic arthritis and 60 had Spondyloarthritis. In psoriatic arthritis patients secukinimab and Adalimumab was given in 3 each and infliximab 200mg was given in 4 and etanercept in one patient. 3 out of 11 patients achieved a drug free remission during this period. And NSAIDS were withdrawn in 9 out of 11 patients. In the remaining Spondyloarthritis group 32 patients received infliximab and etanercept in 6 patients and Adalimumab in 22 patient. A repeat dosing of biological after the first dose was used in only 19 patients. NSAIDS were withdrawn from 44 patients out of the 60 patients.
Conclusion: A single dose of Biologicals achieves good disease control in 73% of patients with SPA.
| PC0122: Biological as a steroid sparing agent in juvenile idiopathic arthritis | |
Nagaprabu V N, Velammal P1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India
Introduction: The management of juvenile idiopathic arthritis(JIA) is quite challenging and the usage of steroids and Nonsteroidal anti-inflammatory drugs (NSAIDS) in pediatric age group has its own limitations. Biologicals have changed the outcome of patients with JIA.
Methods: WE have analyzed the outcome of patients with JIA who had received Biologicals in the period of June 2014 to July 2019 and assessed the need for steroids and the frequency of dosing of Biologicals.
Observations: There were a total of 16 patients who had received Biologicals during this period out of which there were 5 boys and 11 girls. The mean age of the study population was 13 ± 6.23 years. There were 8 patients with enthesitis related arthritis and 4 had systemic onset JIA (SOJIA) and 3 had polyarticular JIA (RF+ve) and 1 had oligoarticular JIA (ANA+ve). Adalimumab was used in 11, Tocilizumab was use in 4 and Etanercept was used in 1. A second and repeated dosing of biological was decided based on the disease activity and was required in only 3. Eight out of these 16 patients achieved total drug free remission in these group and 11 patients were totally weaned off from steroids .NSAIDS.
Conclusion: Biologicals when used appropriately in JIA achieved good drug free remission in 50% of patients and also helped wean of steroids/NSAIDS in more than 60% of JIAs.
| PC0123: The need of regular biological usage in rheumatoid arthritis: A clinical obervation | |
V N Nagaprabu, P Velammal1, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,1PSGIMS & R, Coimbatore, Tamil Nadu, India
Introduction: The management of Rheumatoid arthritis(RA) have changed considerably in this biological era but regular and repeated dosing of Biologicals is often not practically feasible. Methods: We analyzed the case records of patients with RA who have received Biologicals in our centre during the period June 2014 to July 2019 and analyzed the need for regular dosing. Observation: There were a total of 171 patients who were included in this analysis of which there were 139 females and 32 males. The mean age was 49.88 ± 13.61 years. 114 patients had received rituximab 500mg 0& 15 days and only 4 patients during this period needed a repeat dosing. Of which 76 patients were managed without steroids. A low dose of rituximab 100mg weekly for 4 weeks was used in 19 patients but in this only 2 patients we were able to start steroids and one received repeat biological dosing. 8 patients received infliximab and 2 patients were continued on steroids with no one requiring repeat dosing. 3 patients were treated with single dose of etanercept and in 2 we were able to stop steroids.
Conclusion: A repeat dosing of biological were required in only 12.86% of patients ( 22/171) and in 60% (104/171) we were able to stop steroids.
Sabarinath M, Tamilselvam T N, Balakrishnan N, Karthikeyan, Sujatha N, Ramesh R, Mythili S; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India
Background: The three SLE classification criteria, namely American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) 2012 and the new European League Against Rheumatism (EULAR)-ACR 2019 weighted criteria are primarily validated in adults.
Objective: The primary objective of our study is to compare the sensitivity of these criteria in childhood SLE (cSLE). The secondary objective is to compare them with the original validation studies done in adults.
Methods: We conducted a retrospective medical record review study of children with clinical diagnosis of SLE admitted at our tertiary care centre between January 2016 and July 2019. We excluded children with SLE overlap. All three classification criteria were applied to these patients. All three criteria sets were compared against a gold standard of physician diagnosis.
Results: There were 66 patients (84% female) who were diagnosed as cSLE during the study period. The median age at the onset of illness was 15 years. The median duration from the onset of initial symptom to the time taken for diagnosis was 6 months. Sensitivity of ACR criteria and SLICC criteria in childhood lupus was 84% (95% CI 73% to 92%) (n=56) and 95% (95% CI 86% to 98%) (n=63) respectively. The sensitivity of new ACR EULAR weighted criteria was 94% (95% CI 84% to 98%) (n=62). 3 cases didn't satisfy EULAR-ACR weighted criteria as they didn't meet entry criteria (ANA positivity), even though all had the required positive score of >=10.
Conclusion: This study shows that the EULAR-ACR weighted criteria and SLICC has similar sensitivity in cSLE. Both of them have higher sensitivity compared to ACR criteria. The sensitivity of weighted criteria may increase further if entry criteria is not considered. The sensitivities of these criteria in children are comparable with those observed in original validation study done in adults.
| PC0125: Changes in urinary micro-albumin levels after correction of hyperuricemia in patients with gout: An observational study | |
Shweta Nakarmi, Binit Vaidya, Rakchya Joshi; National Center for Rheumatic Diseases, Kathmandu, Nepal
Background: Gout is commonly associated with metabolic syndrome. Strong association between serum uric acid level and microalbuminuria has also been observed in previous studies.
Aim: To observe change in urinary micro albumin after lowering of serum uric acid level in patients with gout.
Methodology: A prospective, observational study was conducted at a tertiary level rheumatic center in Kathmandu, Nepal. Adults diagnosed with gout were enrolled in the study after obtaining informed consent. Sociodemographic profile along with clinical history were recorded at baseline. Serum uric acid levels, spot urinary micro-albumin (MAU) excretion, blood sugar, lipid profile, blood pressure were measured at baseline, 3 months and 6 months follow up. Paired t test was used to compare MAU of the participants.
Results: A total of 778 patients diagnosed with gout were enrolled in this study among whom 97.7% were male with the mean age of 48.8 ± 12.4 years. 36 patients (4.6%) had multiple tophi and 9 patients (0.9%) had polyarticular presentation. Comorbidities like diabetes mellitus, hypertension and dyslipidemia were present in 7.2%, 24.4%, and 47.2% respectively. Most of the patients were overweight and obese with the mean BMI of 27.2 ± 7.6. Around 15.5% patients had microalbuminuria (MAU > 30.0 μg/mg) during presentation. Mean MAU level among those with microalbuminuria was 132.4 ± 124.6 μg/mg. Among 114 patients with high MAU, 35 with concomitant HTN were put on ARBs (telmisartan or losartan). In patients with ARBs, MAU reduced significantly after 3 months of treatment with ARBs. Reduction in MAU in those without ARBs was seen after 6 months follow up and the change was statistically significant.
Conclusions: There is significant reduction in MAU after lowering of uric acid levels in patients with gout.
| PC0126: Relation between cytokines, juvenile idiopathic arthritis and mature of biologics | |
Pragati Datta, Tapas Kumar Sabui; Department of Paediatrics, R.G. Kar Medical College and Hospital, Kolkata, West Bengal, India
Background: Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease affecting children worldwide. Inflammatory cytokines like TNFα, IL-6 playa significant role in the pathogenesis of different subtypes of JIA. Few studies are available on this subject.
Objective: To find out if any relation exists between these cytokines and disease activity in JIA and to find if there is any correlation between nature of cytokines and biologics being used.
Methods: This was a prospective observational cohort study carried out in a tertiary care hospital in Kolkata with 17 JIA cases and 10 healthy controls. Disease activity was measured using JADAS 27 score. Cytokines level was measured at baseline and at 6th month follow-up. Choice of treatment was not based on cytokine profile, rather it was based on availability of drugs and physician's choice.
Results: Levels of TNFα was significantly high in patients with high disease activity at baseline compared to follow up (p<0.001) and controls (p<0.0001) except in Oligoarthritis where TNFα level did not change with disease activity (p=0.4). IL-6 did not show any correlation with disease activity (p=0.07) though mean IL-6 decreased at follow-up. Polyarthritis patients were found to have high levels of TNFα, IL-6 compared to other subtypes. More than 50% patients received biologics(Etanercept, Tocilizumab etc) but levels of TNFα, IL-6 did not reduce significantly in them (p=0.16 and p=0.17 respectively) although disease activity improved in most of these patients.
Conclusion: TNFα correlated with disease activity in polyarthritis and systemic onset JIA, IL-6 did not show any correlation with disease activity. Although most of the patients receiving biologics improved, no correlation was found between nature of cytokine and biologics.
| PC0127: Design of a simple SMS/call based temperature monitoring system for freezers | |
Sonali Waghmare1, Jayakanthan Kabeerdoss2, Anne Tryphosa K3, John Mathew2, Syrpailyne Wankhar3; 1IIT Madras, CMC Vellore, and SCTIMST Trivandrum, 2Departments of Rheumatology and Bioengineering, Christian Medical College, Vellore, Tamil Nadu, India
Background: Large number of bio-specimens like blood, sera, plasma, tissue DNA and RNA, as well as proteins, are stored in freezers under controlled conditions preventing loss of biological nature. However, these specimens can still be damaged because of temperature fluctuations resulting from power failure, compressor failure or any other internal defect. The available freezers can display temperatures and provide alarm signals when alteration in temperature within the lab vicinity. However, such monitors are not useful at night or when no one is in close proximity.
Aim: To design a simple and cost-effective SMS/Call based temperature monitoring system that can remotely send alert signals when faulty conditions occur.
Methods: The device consists of a controlling unit, temperature sensor (LM35/PT100) and GSM module. When the system detects an increase in temperature of +10°C from the reference (-20°C or -80°C) freezer temperature, an alert signals is displayed on the LCD screen and simultaneously SMS and call alerts are sent to the lab in-charge. Data was collected manually every half an hour by monitoring the temperature on the LCD display and that of the respective freezers. Such a system provides a window if a fault occurs, such that necessary actions can be taken before samples incur any damage. The cost of components used in the device is less than Rs.2000.
Conclusion: We developed a cost-effective device which is capable of alert user when the drop in temperature of the freezer by calling and by sending SMS. This device has potential application in preserving valuable biological samples stored in freezers.
| PC0128: Use of biological DMARDs in rheumatology patients in Dehradun, Uttarakhand | |
Kamal Bhatt; None
Background: Use of biological disease modifying agents in rheumatological diseases is rapidly expanding. But their prohibitive cost and fear of adverse effects still preclude their wider use in needy patients. Here is a brief audit of biological use from a rheumatology clinic in Dehradun, Uttarakhand.
Methods: Patient records from January 2015 to December 2018 were analyzed and all patients in whom biological DMARDs were used were included. All patients were assessed clinically, with measures of pain, joint counts, metrology, functional limitation, inflammatory markers, and requirement for NSAID and/ or steroid, but no formal disease activity scores were calculated. Because of small number of patients, no statistical analysis was done.
Results: Biological therapy was used in a total of 60 patients during this four year period, with Spondyloarthritis being the most common indication, and Infliximab the most common drug. The average duration of follow up post bDMARD was 13.7 months with range from 3 to 42 months. Forty-four patients (73 %) had good clinical response. Because of theirs high cost, bDMARDs could only be given either for shorter duration or at long intervals. No major adverse effects including tuberculosis were reported.
Conclusion: Majority of patients had good clinical response without any major adverse effects. bDMARD use is limited by their high cost.
| PC0129: Study of clinical profile, etiology and short- term outcome of interstitial lung disease | |
Shrikant A Mandge, Iravati Waghamre, Deepak Malgutte, Aasna Khan, N T Awad, Yojana Gokhle; Department of Medicine, Rheumatology Services, Lokmanya Tilak Municipal Medical College and Government Hospital, Mumbai, Maharashtra, India
Background: ILD may present as component of CTD or independently. Clinical profile and serological test helps diagnosis of CTD-ILD. ILD is treated with steroids and immunosuppressant. Outcome differs on HRCT pattern (NSIP/UIP), primary condition (Non-CTD) or CTD-ILD, baseline FVC of patient. The literature reports CTD-ILD has better prognosis than Non-CTD ILDs.
Objectives: To study, identify proportion of CTD-ILD and their outcome against Non-CTD-ILD in Indian population.
Methods: It's prospective study. Patients of ILD diagnosed on HRCT/PFT included. Clinical features, baseline 6 minute walk test (6MWT) and PFT (FVC); serology like ANA, RA/ Anti-CCP, Anti-Joe/SCL70 (Suspected Scleroderma), U1RMP (MCTD)done.Patients treated with oral steroids/cyclophosphamide/MMF/perfinidone depending on affordability/fertility issues. Patients classified according to HRCT patterns (NSIP/UIP/MIXED/FIBROTIC-NSIP) and serology/clinical profile (CTDs/Non-CTDs). After 6 months outcome measured with 6MWT and PFTs. Increase in FVC more than 10% and 6MWT distance more than 54m considered significant.
Results: Out of 41 patients enrolled, 3 died, 6 lost follow-up. Average age was 47 years (24-72), females (78%). Average duration of illness was 40 months (6-216). Out of 41cases,26(63%) were CTD- ILDs(Scleroderma 9,MCTD 9,Sjogren 2,Dermatomyositis-Polymyositis 4,RA 1,SLE 1) and 15(37%) non-CTDs. Serology positive in 80%.
HRCT patterns observed NSIP (41.5%), UIP (41.5%), MIXED (9.8%) and FIBROTIC-NSIP (7%).At follow-up, data for FVC and 6MWT was available for 32 patients. Mean difference (baseline minus after 6 months treatment) in FVC significant NSIP>UIP (p=0.0064) and CTDs >NON-CTDs (p=0.004). Data in table-5 showed significant increase in distance covered on 6MWT (p=0.0439).The mean difference in 6MWT significantly greater in NSIP>UIP (P=0.OO59) (unpaired T test values).
Conclusion: Two-thirds of ILD patients were CTD related and had better prognosis.
Anurag Agarwal, Bruno Fautrel1, Baojin Zhu2, Francesco de Leonardis2, Carol Gaich2, Claudia Nicolay2, Zbigniew Kadziola2, Inmaculada De La Torre2, Peter C Taylor3, Mart van de Laar4, Paul Emery5, Roy Fleischmann6; Eli Lilly and Company, Gurgaon, Haryana, India,1University Pierre et Marie Curie, Paris, France,2Eli Lilly and Company, Indianapolis, Indiana, United States,3Botnar Research Centre, University of Oxford, Headington, United Kingdom,4Arthritis Center Twente, Enschede, Netherlands,5Leeds MSK Biomed/Chapel Allerton Hospital, Leeds, United Kingdom,6University of Texas Southwester Medical Center, Dallas, United States
Background: In RA-BEGIN (NCT01711359), baricitinib (BARI) monotherapy demonstrated superior pain reduction and HAQ-DI improvement than MTX.
Objective: To assess pain and HAQ-DI for BARI monotherapy vs adalimumab (ADA), tocilizumab (TCZ), and tofacitinib (TOFA) monotherapy in csDMARD/bDMARDnaive RA patients using MAIC.
Methods: RA-BEGIN BARI 4mgarm patient data were weighted to match baseline characteristics of ADA (PREMIER), TOFA 5mg (ORAL-START), and TCZ 8mg/kg (AMBITION and FUNCTION) arms; MTX arms were also matched. Method of moments determined weights for age, gender, baseline disease scores and baseline outcome variable values. Mean Week24 changes on pain VAS and HAQ-DI (BARI) were adjusted for the characteristics with the weighted linear model and indirectly compared with respective Week-24 TCZ and TOFA and Week26 ADA data. Statistical significance of weighted treatment effect was assessed by bootstrap method. Sensitivity analyses included MAIC with studylevel matching, Bucher's method without matching adjustment, and inclusion of disease duration as additional matching variable.
Results: The mean baseline pain VAS (58.7 to 65.2; 6-month mean change in pain −28.3 to −33.5 [MTX arm]) indicated comparability between trials, with similar HAQ-DI and changes in HAQ-DI for the MTX arm. BARI showed greater improvement (Week 24) over MTX in pain than TCZ, ADA, and TOFA; statistically significant pain improvement was observed for BARI vs ADA and TCZ with all 3 matching Methods:, but only with the Bucher method for TOFA. BARI-treated patients showed significantly greater HAQ-DI Week24 improvement than TCZ and ADA, but not TOFA [Figure 1].
 | Figure 1: N† is the sum of both active arm and MTX arm ESS†-Effecmtive sample size (pain/HAQ-DI) after re-weighting of total N=369 (BARI, n = 159, MTX, n = 210) *P ≤ 0.05; **P ≤ 0.01; *** P ≤ 0.001 Click here to view |
Conclusions: Indirect comparison of studies in cs/bDMARD-naive RA patients, after adjusting for differences in baseline characteristics, suggests greater pain reduction and improved physical function for BARI monotherapy vs TCZ and ADA. There is greater pain reduction for BARI monotherapy vs TOFA, but no differences in improved physical function.
Reference
- Presented at EULAR 2019. Ann Rheum Dis 2019;78 Suppl 2. [doi: 10.1136/annrheumdis-2019-eular.691].
| PC0131: Effects of baricitinib on haematological laboratory parameters in patients with rheumatoid arthritis | |
Anurag Agarwal, Thomas W Huizinga1, Jonathan Kay2, Masayoshi Harigai3, Edward Keystone4, Josef Smolen5, José Rosas6, Paul Emery7, Stephen Hall8, Filip van den Bosch9, Morton Scheinberg10, Jean Dudler11, Ran Liao12, Gabriella Meszaros13, Jane Barry14, Joel Kremer15; Eli Lilly and Company, Gurgaon, Haryana, India,1Leiden University Medical Center, Leiden, Netherlands,2Division of Rheumatology, UMass Memorial Medical Center and University of Massachusetts Medical School, Worcester, Massachusetts,3Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan,4The Rebecca MacDonald Centre For Arthritis, Mount Sinai Hospital, Toronto, Canada, USA,5Medical University of Vienna, Vienna, Austria;6Marina Baixa Hospital, Alicante, Spain,7Leeds MSK Biomed/Chapel Allerton Hospital, Leeds, England, UK,8Cabrini Medical Centre, Malvern, Australia,9University Hospital, Ghent, Belgium,10Albert Einstein Hospital, São Paulo, Brazil,11Hôpital Cantonal, Fribourg, Villars-sur-Glâne, Switzerland;12Eli Lilly and Company, Indianapolis, Indiana, USA;13Eli Lilly and Company, Vienna, Austria,14Eli Lilly and Company, Basingstoke, UK,15Albany Medical College, Albany, New York, USA
Baricitinib (BARI), an oral Janus kinase (JAK)1/2 inhibitor, is used for treating adults with moderate-to-severe rheumatoid arthritis (RA).
Objective: To summarise changes in absolute neutrophil counts (ANC), absolute lymphocyte counts (ALC), platelet counts, and haemoglobin (Hb), and associated adverse events, with BARI treatment.
Methods: Data were pooled from completed Phase 1/2/3 studies and an extension study.
Results: BARI treatment was associated with a decrease in ANC and an increase in ALC and platelets, which returned to baseline with prolonged treatment or treatment discontinuation. Incidence of neutropaenia (<1000 cells/mm3) was rare (<1%) and was not associated with higher risk of overall or serious infections. Lymphopaenia was associated with slightly higher rate of overall infections (Table).More BARI 4-mg (2.3%) compared to placebo-treated (1.3%) patients had platelet count ≥600×109/L. In a 6-study placebo-controlled set (0-24 weeks), 5 BARI 4-mg-treated patients (vs 0 placebo-treated) had “deep vein thrombosis” (DVT) and/or “pulmonary embolism” (PE). Incidence rate of overall and serious DVT/PE in ALL BARI-RA set remained low at 0.5 and 0.3 per 100 patient-years, respectively.
With long-term BARI treatment, Hb levels decreased transiently before returning to levels slightly higher than baseline at Week 52. Incidence of severe treatment-emergent (TE) shifts in Hb (grade <3 to grade ≥3: <8 and ≥6.5 g/dL) was low across all treatment groups (<0.5%).
Conclusions: No associations were observed between ANC decrease and infections or thrombocytosis and DVT/PE. BARI treatment was not associated with an increased incidence of erythropaenia-related events or anaemia compared to placebo. Few patients interrupted/discontinued BARI due to TE laboratory abnormalities.
Previously presented at BSR (2018).
 | Table 1: Infection by worst neutropaenia and lymphopaenia CTCAE Grade in 6-Study placebocontrolled* period up to week 24 Click here to view |
| PC0132: A network meta-analysis to evaluate the efficacy of baricitinib and other treatments of rheumatoid arthritis in patients who are inadequate responders to methotrexate | |
Anurag Agarwal, J S Smolen1, P Emery2, J Dudler3, C Zerbini4, W Fakhouri5, C Nicolay6, I de la Torre7, G Burmester8; Eli Lilly and Company, Gurgaon, Haryana, India,1Medical University of Vienna, Vienna, Austria,2Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, England, UK,3HFR Fribourg Hopital Cantonal, Fribourg, Villars-sur-Glâne, Switzerland,4Centro Paulista de Investiga Sao Paulo Clinica, Sao Paulo, Brazil,5Eli Lilly and Company, Windlesham, Surrey, UK,6Eli Lilly and Company, Lilly Deutschland GmbH, Bad Homburg, Germany,7Eli Lilly and Company, Indianapolis, Indiana, USA,8Department of Rheumatology and Clinical Immunology, Charité - University Medicine Berlin, Berlin, Germany
Background: Baricitinib (BARI) is used for treating adults with moderate-to-severe rheumatoid arthritis (RA).
Objective: To assess the comparative effectiveness of BARI 4-mg and other targeted synthetic/biologic diseasemodifying anti-rheumatic drugs in RA patients with inadequate response to methotrexate (MTX-IR).
Methods: A systematic literature review (SLR) of randomized controlled trials (RCTs; Phase 3) of interventions was conducted (1999 to 2017) in Medline, Medline In-Process, Embase, Biosciences Information Service, the Cochrane Library, and trial registers. Network meta-analyses (NMAs) of RCTs reporting American College of Rheumatology (ACR) response data were conducted using Bayesian mixed-treatment comparisons. We present main Results: for the 24(±4)week timepoint (fixedeffects simultaneous models).
Results: 24 trials met the SLR inclusion criteria. Analyses, using BARI RA-BEAM trial data, showed BARI 4-mg (Background MTX) is more effective than adalimumab (ADA) 40-mg (EOW; odds ratio [OR] 1.33; 95%Credible Interval [CrI] 1.01-1.75), abatacept (ABA) 10-mg (IV/4 weeks; OR 1.47; 95%CrI 1.02-2.13), and infliximab 3-mg (IV/8 weeks; OR 1.61; 95%CrI 1.12-2.27), for ACR20. No differences were found on ACR50; BARI 4-mg (Background MTX) was found to be more effective than ADA 40-mg (OR 1.39; 95%CrI 1.02-1.89), ABA 10-mg (OR 1.85; 95%CrI 1.09-3.23), rituximab (RTX) 1000-mg (OR 2.38; 95%CrI 1.10-5.00) and 2000-mg (OR 2.44; 95%CrI 1.04-5.56) for ACR70. Bari 4-mg (Background MTX) showed better Results: than etanercept monotherapy (50 mg/week or 25 mg/biweekly; OR 2.27; 95%CrI 1.04-5.26) for ACR20 and RTX 1000-mg monotherapy for ACR20/ACR70 (OR 1.82; 95%CrI 1.023.13)/(OR 2.70; 95%CrI 1.04-7.14), respectively. Sensitivity analysis including 10 additional trials with up to 20% patients with prior biologic use allowed comparison versus tofacitinib (TOFA), showing BARI 4mg (Background MTX) is more effective than TOFA 5-mg (BID) monotherapy for ACR20 (OR 1.92; 95%CrI 1.32-2.86).
Conclusion: The analyses support BARI as an efficacious treatment option for moderate-to-severe RA patients with MTX-IR.
Previously presented at ISPOR, Barcelona (2018).
| PC0133: A study of clinico-serological parameters, management and outcome of scleroderma interstitial lung disease | |
Irawati Waghmare, Shrikant Mandge, Lalana Kalekar, Dnyaneshwar Halnor, Ruchita Dhurat, Yojana Gokhale; Department of Medicine, Rheumatology Services, Lokmanya Tilak Municipal Medical College and General Hospital, Mumbai, Maharashtra, India
Background: Scleroderma-ILD is leading cause of mortality and morbidity.It is reported in 90% patients(HRCT) and 40-75%(PFT).Reported literature regarding outcome of scleroderma-ILD with treatment with mycophenolate and cyclophosphamide is significant (p<0.01),without statistical difference in outcome between the two.
Objectives: To compare outcome with cyclophosphamide and mycophenolate in scleroderma-ILD.
Methods: It is a prospective study.Patients with scleroderma(ACR criteria 2013)with ILD(HRCT/PFT proven)were included.Baseline HRCT,PFT,DLCO,six-minute walk test and 2D-echocardiography were performed.Selection of treatment was as per patient's affordability for mycophenolate.
Comparison was made after six months of monthly pulses of cyclophosphamide at dose of 600mg/m2 versus MMF daily 1.5-2g(as tolerated).More than 10% improvement in FVC,15% in DLCO,and an increase in 54m distance on 6MWT was considered significant outcome.
Results: Total 46 patients with scleroderma-ILD were studied.80.4% were females.Study population had an average age of 39 years (range:15-61 years) and average duration of illness of 49 months (range:3-144 months).As per HRCT,NSIP=54.3%,UIP=41.3% and 4.3% had normal finding(only low FVC).
35 patients were in cyclophosphamide group and 11 in MMF group.4 patients were lost to follow up in each group.
Baseline mean FVC was 56.2%,DLCO 42.53% and 6MW distance was 311.42 m.The average increase in FVC was 9.45% with treatment. Summarises the outcome in FVC, DLCO and 6MWT in both treatment groups.
Conclusion: Significant improvement was seen in FVC in 12 patients (p=0.035),DLCO in 4 patients (p=0.004) and 6MW distance in 2 patients(p=<0.01).
There was no significant statistical difference between MMF and cyclophosphamide groups with regard to outcome(p=0.593).
| PC0134: Demographic and clinical presentation of rheumatoid arthritis | |
Agarwal N, Dubey S, Malaviya A N, Sharma S, Nagpal PS*; aIndian Spinal Injuries Centre, New Delhi,bAmity Institute of Virology and Immunology, Amity University, Noida, Uttar Pradesh, India
Introduction: Rheumatoid Arthritis (RA) is the most common inflammatory arthritis seen in clinical practice.Prevalence of RA is estimated as 0.5% to 1% worldwide and in Indian if found to be 1%. The purpose of this study was to investigate the gender ratio, the age of onset, family history, smoking status and the main clinical symptoms.
Materials and Methods: 100 Patient with RA classifiable according to ACR/ELUAR criteria, were recruited for the study. Data were collected at each visit of the patient to the rheumatology out-patients clinic of this tertiary health care facility in New Delhi, India.
Results: Out of the 100 patients, 84% were female and 16% were male with a female to male ratio of 5.25:1. The average age of the patients was was 48.02±10.63 years. The peak age of onset of the disease was between 30-50 years. Depending upon the disease activity score-28 (DAS-28) at the first presentation to the rheumatology clinic, the patients could be classified into 3 groups: namely: High Disease activity (32%), Moderate disease activity (49%) or, Low-disease activity/Remission DSA (19%). DAS28 was found to be in positive correlation with Body-Mass Index (r=0.347, p=0.000). 83% patients were found to be sero-positive and 17% were sero-negative for the auto-antibodies.
38% patients had a positive family history, of whom 84% had first degree relative(s) with the disease. Out of the 84 female patients, 45% experienced miscarriage during the first trimester of their pregnancy. Additionally, 4.7% females had death of their newborns within three days of their birth.
Conclusion: The study found a significant correlation between BMI and DAS28. Females with RA may require a close monitoring during pregnancy and peripartum period.
| PC0135: Clinical and serological characteristics of anti-nucleosome antibody in systemic lupus erythematosus | |
S Rajesh; Kerala Institute of medical Sciences, Thiruvananthapuram, Kerala, India
Background: Anti nucleosome antibody though not routinely checked is a well established antibody with correlation with clinical activity and with other serological markers.
Aim: Retrospective study from a cohort of 400 SLE patients, to assess the clinical and serological correlation of antinucleosome antibody in those patients in whom it was detected.
Methods: Patients who had antinucleosome antibody positive were collected from electronic medical record and the clinical and serological characteristics was assessed. Anti ds DNA was done by ELISA, Antinuclesome antibody was detected by Euroimmune ANA profile immunoblot assay (definite and strong positive patterns), complement assay was done by nephelometry. SLE disease activity was evaluated by using SLE-Disease Activity Index (SLEDAI) score.
Results: 52 patients who were positive for antinucleosome antibody were assessed. Predominant system that was involved was mucocutaneous followed by musculoskeletal system. All patients studied were in an active stage of disease and were untreated, of which 5 patients had renal biopsy-proven kidney involvement, which was categorized as lupus nephritis (LN) and rest did not show any renal manifestations (SLE without LN). Hematological involvement was in the form of pancytopenia and secondary antiphospholipid syndrome was present in one third of cases. Neurological involvement and vasculitis was a minority presentation in this cohort. All but 4 off these patient were anti ds DNA positive showing a significant correlation and there was a significant inverse correlation with complement c3 levels.
Conclusion: Anti-nucleosomal antibody detection in our cohort showed predominant extrarenal manifestations and was useful as an additional disease activity marker to other laboratory tests. There was significant correlation with Anti ds DNA positivity and low c3 levels.
| PC0136: Use of photo- protection in patients with systemic lupus erythematosus: Awareness, attitude and behaviour: A study by rheumatology nurse counsellors | |
Baghel SS1, Thakran R2, Messi C2, Yadav V2, Kapoor S 3, Garg SR3, Vivekanand4, Malaviya A N5; 1 Senior Rheumatology Nurse Counselor, 2 Rheumatology Nurse, 3Consultant Rheumatologist, 4Allied Health Professionals, 5Head of the Department of Rheumatology, Indian Spinal Injuries Centre Superspeciality Hospital, New Delhi, India
Background: Photosensitivity is a common manifestation of SLE, affecting an estimated 57-73% of patients. Photoprovocation tests have shown that ultraviolet irradiation (UVA and UVB) induces cutaneous lesions in these patients. Modern sunscreens that block UV rays are effective in reducing disease flares. Some reports have also suggested that Sun exposure can lead to organ damage e.g. lupus kidney disease.
Objectives: To assess the awareness and compliance of sunscreen use amongst patients with SLE and to determine whether photoprotection advice was provided at diagnosis and follow visits.
Methods: All SLE patients attending the rheumatology clinic, willing to participate in the survey, were enrolled in this study. All the patients were counselled about to avoidance of excessive Sun exposure with continuous photoprotection through physical measures such as protective clothings and daily application of broad-spectrum sunscreens. At every follow-up visit, the patients were repeatedly counselled about avoiding Sun exposure and the use of sunscreen. All the relevant information was collected in a pre-designed form.
Results: 78% of patients had photosensitivity and 96 %were aware that exposure to sunlight was related to skin exacerbation.72% of the patients had at least annual exposure to tropical sunlight. However, only 54% of patients reported using sunscreen consistently and 26% used sunscreen occasionally.45% used sunscreen with 50 SPF, 18% used 30 SPF and 17% were not aware of what SPF they were using. 20% did not use sunscreen even in when the Sun exposure was expected.
Conclusion: Although there was excellent awareness about photoprotection amongst patients with SLE.Only 54 % using them regularly and only 45% using SPF >50 the study showed that all the patients would benefit from education and counseling about appropriate photoprotective behavior, in particular, the need for consistent use of high factor sunscreen preparations together with the use of other protective measures.
| PC0137: Safety of baricitinib in patients with moderately to severely active rheumatoid arthritis: A subanalysis of the Indian population from phase 2/3 clinical studies | |
Jyotsna Oak, Syamasis Bandyopadhyay1, Sundeep Upadhyaya2, Anurag Agarwal3, Rohit Arora3, Subhashini Arthanari4; Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra,1Apollo Gleneagles Hospital, Kolkata, West Bengal,2Indraprastha Apollo Hospital, Delhi,3Eli Lilly and Company, Gurgaon, Haryana, India,4Eli Lilly and Company, Singapore
Background: Baricitinib, an oral selective inhibitor of Janus kinase 1/2, is approved in more than 50 countries for the treatment of moderately to severely active rheumatoid arthritis (RA) in adults.
Objective: To provide a comprehensive integrated summary of safety of baricitinib in Indian patients with RA who participated in global Phase 2 and 3 studies.
Methods: Integrated safety analyses for the Indian population were reported using pooled data from three completed studies (Phase 2, JADA; Phase 3, JADZ and JADX) and one ongoing long-term extension study (Phase 3, JADY). Patients were randomised to receive baricitinib 2-mg, baricitinib 4-mg, or placebo. The percentage of patients experiencing any treatment-emergent adverse events (TEAEs), serious adverse events (SAEs), and temporary or permanent baricitinib discontinuation were reported. Exposure-adjusted incidence rates (EAIRs) per 100 patient-years were calculated.
Results: Of 1469 patients, 131 (9%) patients were enrolled from India. No deaths were reported among patients from India (India: 0%; overall: 0.1%). Four patients in the Indian population reported AEs that led to permanent discontinuation of the drug. The EAIRs for TEAEs were anemia (India: 3.1; overall: 2.2) and herpes zoster (India: 2.1; overall: 2.8). The most common SAEs in both populations were infection and infestation (India: 2.3%; overall: 3.8%). One followup case of bone tuberculosis was reported. No cases of pulmonary embolism or deep vein thrombosis were reported in the Indian population [Table 1].
 | Table 1: Safety parameters in patients with moderately to severely active rheumatoid arthritis (Indian and overall population) Click here to view |
Conclusion: Baricitinib was well tolerated by Indian patients with moderately to severely active RA, and the Results: were consistent with the overall population included in the randomised controlled studies.
| PC138: Clinical and serologic profile of patients diagnosed as idiopathic inflammatory myositis | |
Deepak Rath, Pradyot Sinhamahapatra, Alakendu Ghosh; Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata, West Bengal, India
Introduction: Idiopathic Inflammatory myositis are a heterogeneous group of systemic autoimmune rheumatic disorders, with 2 peaks of occurrence. Auto-antibodies are commonly detected in upto 80% of the cases.
Methods: A retrospective chart analysis was undertaken for 19 patients admitted to the Rheumatology Department between 01 August 2016 – 31 July 2019, who were diagnosed as Idiopathic Inflammatory Myositis.
Results: There were 12 females and 7 males. Juvenile dermatomyositis was diagnosed in 1 male only. The mean age at diagnosis was 34 years. Mechanic's hand was seen in 3. Typical rash of dermatomyositis was seen in 12. Calcinosis cutis was seen in 1. ANA positivity was reported in only 4, but their ANA profile was negative. 4 patients had Ro52 positivity on ANA profile, while 1 patient had RNP/Sm positivity. 8 patients were Mi2 positive, while 6 were Jo1 positive, PL 7 positive = 2, PL 12 positive, OJ positive and Pm/Scl 75 positive was noted in 1 each. The lowest mean MMT-8 score was seen in Mi2 Positive individuals (score: 67) Patient with Pm/SCl 75 positivity did not have any muscle weakness. ILD was seen in 8/19 patients. Cardiac involvement was noted in 3 patients. MRI of the muscle was done in 5 patients, which had shown evidence of Patchy hyperintensities in the proximal group of muscles. 2 patients had normal EMG, while the remaining 17 patients had pattern suggestive of myopathy.
Discussion: Patients with IIM are diagnosed on a constellation of clinical and laboratory parameters. Patients are afflicted at a mean age of 35. MRI shows patchy hyperintensities in the proximal muscle groups of the patients. EMG shows myopathic pattern in these patients. Many patients are unwilling for a muscle biopsy.
Conclusion: Indian patients of IIM are afflicted a decade early. Mi2 positivity is more common than Jo 1 positivity.
| PC0139: Short term and durable remission in rheumatoid arthritis: Differences and determinants | |
Sanchaita Misra, Sumantro Mondal, Satarupa Dutta1, Dipanjan Bhattacharjee, Sulagna Chatterjee, Ayindrila Saha, Sudipta Chatterjee, Pradyot Sinhamahapatra, Debasish Lahiri, Alakendu Ghosh; Department of Rheumatology, SSKM Hospital, Kolkata, West Bengal,1National Institute of Animal Biotechnology, Hyderabad, Telangana, India
Introduction: Remission is the ultimate treatment goal in the management of patients with rheumatoid arthritis (RA), more precisely a sustained remission. Subclinical synovitis can be detected by Ultrasonography (USG) in patients with RA who are in remission and it may predict disease flare. Perturbation of the levels of cytokines and angiogenic markers can be seen even in the remission state.
Aim: This study was intended to find the levels of pro inflammatory and angiogenic mediators among the short term and sustained remission RA patients and also to depict the distribution of US Synovitis score between these two groups. The clinical and laboratory determinants of sustained remission were also evaluated.
Methods: Thirty four RA patients who are in remission, fulfilling both CDAI and DAS28 criteria were recruited . Based on the duration of remission two groups were divided as follows: 1. Sustained remission (remission duration of ≥ 6months) and 2. Short term remission (remission duration < 6 months). Demographic and clinical data were collected. Every patient underwent USG evaluation of 14 joints and grey scale Synovitis (GSS) scoring was done. Following cytokines and angiogenic markers were measured by ELISA (TNF-α, IL-17, IL-1b, ILand VEGF).
Results: Increased pro inflammatory cytokines and angiogenic markers were found in short term duration remission patients [Table 1].High GSS score was observed in short term remission patients with positive correlation with IL-17 and VEGF (r=0.5, r=0.7). Negative correlation has been found between DMARDs initiation gap and duration of remission(r=-0.4).
 | Table 1 Click here to view |
Conclusion: Upregulated pro-inflammatory and angiogenic mediators and high USG GSS score can be seen in short term remission of RA pointing towards an ongoing inflammatory process. VEGF and IL 17 are associated with sub clinical synovial proliferation in remission state. Early DMARD initiation should be the therapeutic strategy to achieve sustained remission.
| PC0140: Premature atherosclerosis in rheumatoid arthritis is uncommon in patients residing in parasite endemic area | |
Meghanad Meher, Manoj Kumar Parida, Bidyut Das AIIMS BBSR, SCBMCH CTC, SCBMCH CTC
Background: Parasites are known to downregulate immune response through modulation of both innate and adaptive immune system in rheumatoid arthritis. Atherosclerosis has been found to be independent cardiovascular risk factor in rheumatoid arthritis and has been observed in 10-20% of cases.
Aim of the study: Assessment of CIMT (a standard screening test to detect atherosclerosis) in patients of rheumatoid arthritis residing in parasite endemic area.
Methodology and Results: In our cross sectional study we enrolled 58 patients of rheumatoid arthritis diagnosed by ACR-EULAR 2010 criteria. We excluded patients having diabetes mellitus, hypertension, dyslipidaemia, hypothyroidism, CKD and smoking. CIMT is measured by B mode USG scan by using 3-12 mega Hz probe on grey scale over bilateral common carotid artery. Out of 58 patients 48 were female and 10 were male. Average age was 44.1±11 years. Average duration of disease was 4.1±1.2 years. Rheumatoid factor was positive in 48 patients and negative in 10 patients. Average ESR was 73±32. Average CRP was57±42. Average DAS 28 score was 6.5±0.92. Average CIMT in Rheumatoid Arthritis patients was 0.65±0.10mm as compared to control value of 0.57±0.30mm. 3 out of 58(5.17%) patients had high CIMT value (i.e.>0.80mm).
Conclusion: Prevalence of atherosclerosis in rheumatoid arthritis is uncommon in patients residing in parasite endemic area despite a chronic inflammatory state. However a study on a larger number of patients will validate this observation.
| PC0141: To evaluate prevalence of low bone mineral density in otherwise normal premenopausal Indian women: A cross-sectional study | |
Ankur Dalal; The Sarvajanik Medical Trust Hospital, Rampura, Surat, Gujarat, India
Background: Osteoporosis and osteopenia are major public health problem and growing concern in both developed and developing countries worldwide including India. This entity is of utmost important; however, routine screening is not a common practice in India due to lack of awareness, facilities and cost-effectiveness in measuring bone mineral density (BMD).
Objectives: Evaluation of prevalence of low BMD in otherwise normal premenopausal Indian women by screening with peripheral-dexa (p-DEXA) heel BMD measurement.
Methods: Total 66 premenopausal adult women voluntarily attended free screening camp at tertiary care hospital were included in the study after applying inclusion and exclusion criteria. Peripheral heel BMD was measured using p-DEXA bone densitometer. T-score was calculated as per WHO equivalent for heel BMD [1]. Data were collected and analyzed using descriptive statistics.
Results: Mean age of study population was calculated as 34.32 ± 16.09 years. Overall prevalence of osteoporosis observed was 19.69% while of osteopenia was 39.39%. Prevalence of low BMD (osteopenia plus osteoporosis) in otherwise normal premenopausal Indian women observed in study was 59.09% [Table 1].{Table 31}
Conclusion: Prevalence of osteoporosis and osteopenia is significantly high even in otherwise normal premenopausal Indian women. Its screening by measuring peripheral heel BMD using p-DEXA in routine clinic practice is convenient, cost-effective and quite useful in planning preventive strategies to improve bone health and thus reducing the future fracture risk.
Reference
- World Health Organization. Assessment of Fracture Risk and its Application to Screening for Postmenopausal Osteoporosis: Report of WHO Study Group. Technical Report Series 843. Geneva, Switzerland: World Health Organization; 1994.
| PC0142: Clinical profile of primary antiphospholipid syndrome | |
Arul Rajamurugan; Madurai Medical College, Madurai, Tamil Nadu, India
Background: Antiphospholipid syndrome ( APS ) is a systemic autoimmune disorder characterised by arterial and venous thrombosis and / pregnancy morbidity in the presence of antiphospholipid antibodies. APS occurs as primary condition or it can occur with SLE or other autoimmune diseases.
Aim: To study the clinical profile of primary APS in patients attending Rheumatology OP. Methods: A retrospective analysis of the records of the patients fulfilling the diagnostic criteria of primary antiphospholipid syndrome who attended rheumatology op over a period of 2 years from 2017 – 2019 was done. After thorough Clinical examination, haematological, immunological studies were done. Results: Out of 20 patients studied,1 presented with catastrophic APS, 8 had deep venous thrombosis; 5 had cortical venous thrombosis; 1 had chronic IVC thrombosis; 1 had isolated thrombocytopenia; 4 presented with pregnancy morbidity. Out of all 12 were female and 8 were males. Manifestation incidence(%). DVT lowerlimbs 40, Cortical venous thrombosis 25, IVC thrombosis 5, Foetal loss 20, Thrombocytopenia 5, Catastrophic APS 5.
Conclusion: From this study, DVT has higher proportion in primary APS followed by pregnancy morbidity. There is a female preponderance.
| PC0143: Spectrum of connective tissue disease in a community based center | |
Bharat Veer Manchanda, Anuradha Venugopalan, Rahul Patil, Arvind Chopra; Rheumatology Fellow,
Introduction: Undifferentiated CTD and overlap CTD has been described since long but not sufficiently enough to allow classification based on the generally accepted criteria. Whether the CTD-U represents distinct clinical entities or the presentation of well-defined connective tissue disease (CTD) is still a matter of debate.
Methods: This is a cross-sectional retrospective study of patient data extracted from the CRD database. Study period:2016-2018. Patients with the clinical diagnosis of undifferentiated features (CTD-U) and/or overlap of two CTD(OCTD) analyzed and presented.
Results: 192 patient records retrieved with diagnosis of CTD-U or OCTD. The basis of diagnosis was predominantly clinical. Mean age was 38 years (range:13-77 years). Male: Female ratio 1:9. Mean disease duration was 6 years (range: 2 months-30 years). Tobacco consumption was in 26 patients whereas 14 patients had a habit of taking regular alcohol. 128 patients had musculoskeletal involvement as initial symptom at onset in which 89% were polyarticular. CTD-U diagnosed in 78 patients; OCTD:55; Mixed-CTD:32; Systemic Lupus Erthyematosus:15; Progressive Systemic Sclerosis:9; Dermatomyositis:2; Autoimmune Thyoiditis:1. Co-morbidity was seen in 32 patients (Diabetes=1; hypothyroidism=20; hypertension=11; asthma=1). Systemic involvement and laboratory features.
Conclusion: Our study shows that there is little doubt that the overlap or undifferentiated diseases represent a diverse clinico-serological spectrum characterized by a mild clinical picture which changes little over time. Also, these patients show limited autoantibody repertoire with a low incidence of disease-specific autoantibodies. Early recognition and unusual associations of symptoms is crucial in management of such patients.
| PC0144: Co-existence of ra and gout: A rerospective analysis from a community referal centre | |
Bharat Veer Manchanda, Anuradha Venugopalan, Abraham Mohan, Arvind Chopra; Rheumatology Fellow
Introduction: Gout and RA are common inflammatory arthropathies, with the prevalence of RA is 0.67(rural) & 0.32(urban) and that of gout 0.13(rural) and 0.06(urban) (Chopra et al,2009). For reasons not understood the RA-Gout coexistence in published literature is UNCOMMON when both disorders are not uncommon in the community.
Methods: Data extracted from referral database in CRD maintained since 1996. This was a cross-sectional retrospective study with prospective follow-up. Study period was 2001-2018. The basis of diagnosis for RA and gout was clinical. Synovial fluid aspiration done to demonstrate crystal in few difficult cases.
Results: From 53498 case records during study period, RA alone was diagnosed in 17480(32.7%); 784(1.5%) only gout and 54(0.1%) diagnosed with co-existence of Gout & RA; Male:Female=5:1; mean age 52years (range:18-77years). 32 patients fulfilled ACR Criteria(2010) for Rheumatoid Arthritis and ACR criteria(2015) for gout. 31.5% used tobacco; 25.9% consumed regular alcohol. 7.4% reported classical podagra. Classical tophi seen in 9 patients; single case presenting chalky white discharge from tophus. Articular pattern was pre-dominantly polyarticular. All had significant early morning stiffness(>30-45 minutes). MTP were commonest joints involved followed by ankle. Radiological evidence of gout seen in 28(51.9%) & of RA in 9(16.7%); 21 had mild-to-no-erosive disease on x-ray. Hyperuricemia in 75%(mean serum uric acid=8.3 mg/dl); RF &/or Anti-CCP positive in 51%. Two had renal calculi; none had renal insufficiency/failure; co-morbidity in 12 patients (Hypertension:6;IHD:1;Obesity:4;Diabetes Mellitus:1). Standard RA treatment and Allopurinol was the treatment in all cases. 52 cases have been followed-up (mean follow-up period:4years).
Conclusion: Coexistence of gout & RA was often clinical but satisfied ACR criteria for both in 2/3rd patients. It may be prudent to speculate that this is often a missed combination by rheumatologists. Whether co-existence of gout in RA increases bone damage & kidney, needs to be evaluated prospectively in a larger cohort.
| PC0145: Early rheumatoid arthritis is an advantage with community based rheumatology centers: Lesson from CRD, Pune | |
Rahul Patil, Anuradha Venugopalan, Arvind Chopra; Rheumatology Fellow
The need for early diagnosis and early treatment in Rheumatoid arthritis (RA) is critical and this opportunity is usually lost in tertiary rheumatology centres. As a community based referral centre we were keen to know that with popularity and time, are we seeing an increase in the proportion of early RA (ERA) cases.
Methods: The current study is a retrospective study of patient records from Jan 2015-Dec 2018 extracted from a comprehensive database, maintained since 1996. The first visit profile of patients diagnosed with early RA(ERA) within 12 months of onset of symptoms is being presented.
Results: Among 64,689 first(initial) visit patient records maintained since 1996, 30,878(47.7%) have been diagnosed as RA. Since 2015–2018, among 4140 RA, 915(22.1%) have been diagnosed within 12 months of disease onset.
Overall Female:Male ratio in the early RA(ERA) cohort was 4.6:1. Median age 45years. Although almost equal proportion of patients came from the rural & urban areas, the larger percent of patients were walk-in(87.8%) as compared to those who were referred by physicians. The year wise data, baseline clinical and laboratory features.
Conclusion: Early RA needs immediate specialist assessment and care. The proportion of ERA in our center has been increasing over the years; a lesser proportion showing presence of erosions at their first visit to our center. A significant number of patients were walk-in rather than being referred by primary care physicians. The awareness of the disease and the necessity to approach specialists or tertiary care was as good in rural as among the urban population.
| PC0146: A comparative study between low dose versus standard dose rituximab in patients of rheumatoid arthritis in a tertiary care center of North Bengal | |
Saikat Singh, Pasang L. Sherpa, Biswadip Ghosh; North Bengal Medical College and Hospital, Siliguri, West Bengal, India
Background: Rheumatoid arthritis is a very common chronic poly-arthritis which can lead to joint deformity and restriction of activities. Rituximab is a chimeric monoclonal antibody directed against CD20 molecule present on mature B cell surface.
Objective: In this study, we aimed to prove non inferiority of low dose rituximab(2*500mg) compared to standard dose(2*1000mg) as assessed by treatment response at 6 weeks and 12weeks using 2 parameters DAS28ESR and CDAI.
Methods: 30 patients, who failed to respond to methotrexate alone or a combination of DMARDs, were included in the study. They were divided in 2 groups randomly. 1 group received 2 doses of rituximab 500mg each and the other group received 2 doses of 1000mg each at 2 weeks interval.
Results: We found that in low dose group mean DAS28ESR scores at beginning, 6 weeks and 12 weeks were 6.89, 4.58 and 3.25 respectively; CDAI scores for the same group were 53.06, 25.2 and 14.13 respectively. Both parameters showing significant improvement; p value for DAS28ESR scores were 0.00001 for 6 weeks and 0.0001 for 12 weeks and p value CDAI scores were 0.01 for 6 weeks and <<<0.05 for 12 weeks. In the standard dose group, DAS28ESR scores at beginning, 6 weeks and 12 weeks were 7.0, 4.85 and 3.53 respectively; CDAI scores for the same group were 53.33, 26.33 and 15.06 respectively, showing significant improvement with p values of 0.003 and 0.00007 for DAS28ESR for 6 weeks and 12 weeks respectively and 0.006 and 0.007 for CDAI for 6 weeks and 12 weeks respectively. However, there was no statistically significant difference among the two study groups as indicated by p value >0.5(0.67 for DAS28ESR and 0.12 for CDAI).
Conclusion:
Low dose rituximab is non inferior to standard dose rituximab in efficacy in rheumatoid arthritis upto atleast 12 weeks.
| PC0147: Outcome at a median period of 5 years on methotrexate and prednisolone therapy in patients with Takayasu's Arteritis | |
Koshy Nithin Thomas, Avinash Jain, Durga Prasanna Misra, Amita Aggarwal, Able Lawrence, Vikas Agarwal, Latika Gupta, Ramnath Misra; Department of Clinical Immunology and Rheumatology, SGPGI, Lucknow, Uttar Pradesh, India
Background: There is paucity of data on long term outcome of TA patients on conventional immunosuppressants. We aimed at analysing outcome (response, remission and refractory disease) of TA patients on Methotrexate using validated outcome measures.
Methods: Patients who fulfilled ACR criteria of TA with a minimum 1-year follow up on Methotrexate and prednisolone were retrospectively analysed. ITAS, ITAS-A and TADS score were retrieved at baseline and last follow up. ITAS ≥ 2 and ITAS-A ≥ 3 (with ITAS at least 1) were defined as active disease. Refractory disease was defined as no response or angiographic progression.
Results: Thirty-four TA patients (24 female) with median (IQR) age of 27 (20.75-39) years and disease duration of 18 (11-49) months were included. The median(IQR) follow-up period was 4 (2-5) years. Methotrexate therapy resulted in remission in 23/34 patients. Median (IQR) ITAS, ITAS-ESR(A) and TADS at baseline were 13 (7.75 – 16.25), 15 (9.5 – 18) and 1(0-3.25) respectively, and were 0, 2 (1-3) and 7.5 (6-9.25) at last visit respectively. All but 4/34 patients showed accrual of damage with median change in TADS 6 (3-8). Median dose (IQR) of Methotrexate at baseline was 15 (10-15). Methotrexate was switched to Azathioprine and MMF in 6 and 1 patients respectively due to unresponsiveness after median period of 2 (1-2 years). Six out of these 7 patients went into remission. Three patients had refractory disease (including 1 in whom Methotrexate was changed). Two patients had relapse on Methotrexate after 1 and 2 years of treatment. Six patients were off steroids at last visit. Radiographic progression was seen in 9/10 patients. Revascularisation procedures were done in 6 patients. No predictors for remission, refractory disease could be identified.
Conclusion: Methotrexate and corticosteroid effectively provide remission in the majority (67%) of patients with Takayasu' arteritis.
| PC0148: Assessment of safety and efficacy of apremilast in psoriatic arthritis patients: Single centre study | |
Rahul Babu, Reshma Reji, K C Shanoj, Padmanabha Shenoy; Dr Shenoy's Care, Centre for Arthritis and Rheumatism Excellence, Chennai, Tamil Nadu, India
Background: Psoriatic arthritis (PsA) is a chronic inflammatory arthritis seen in 30 to 40 % cases of psoriasis. Treatment with conventional disease modifying anti rheumatic drugs (cDMARDs) as monotherapy or in combination therapy have not yielded satisfactory. Results: Our study analyses the effects of the novel agent, Apremilast, in both skin and joints.
Objectives: To analyse the efficacy and safety of Apremilast in patients with PsA.
Methods: A retrospective observational assessment of experience with Apremilast in PsA patients from a single centre. Data were collected during the period from Feb 2018 to July 2019 using electronic medical records. All patients initiated on Apremilast 30 mg BD were included in the study. Their demographic details, co morbidities, baseline disease activity (tender joint count (TJC), swollen joint count (SJC) and Psoriatic arthritis severity index (PASI)) were collected. Their current therapy status i.e., co prescribed DMARDs/Steroids were also included.
Results: A total of 157 patients were selected. Apremilast was discontinued in 13 patients due to intolerance. 26 patients had low disease activity, 7 had insufficient data and 2 had only psoriasis. So in the effective sample size of 109 patients, 42 (38.5%) were found to be females. Baseline mean age and disease duration were 48.84±10.30 & 50(42, 55) respectively. A significant change from baseline disease activity was found at the end of 3 months.The change in TJC, SJC and PASI were not significantly different in the early PsA and late PsA groups. A total of 25 patients were not tolerating optimum dose of Apremilast. The most common adverse events were gastric intolerance and diarrhoea. Detailed Results: of the study are depicted.
Conclusion: Apremilast was found to be an effective option for controlling inflammation in both skin and joints in PsA patients.
| PC0149: Clinical profile of patients with anti synthetase syndrome admitted to Dept. of Rheumatology IPGME&R and SSKM Hospital, Kolkata | |
Basil Paul Kunnathu, Alakendu Ghosh; Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata, West Bengal, India
Introduction: Anti-Synthetase Syndrome (ASS) is an uncommon autoimmune disease characterized by the presence of antiaminoacyl tRNA synthetase (anti ARS) antibodies along with interstitial lung disease, inflammatory myositis, arthritis, fever, mechanics hand and Raynaud's phenomenon. The clinical presentation of ASS is variable and partly depends on the type of antiARS antibody present.
Methods: A retrospective analysis of the case records of patients with Anti Synthetase Syndrome, who were admitted to the Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata between October 2014 and February 2019.
Results: Nine patients diagnosed with Anti Synthetase Syndrome were studied. There were five females and four males. The mean age at diagnosis was 34.4 years. Four patients were Jo1 positive, while three were PL 7 positive, one with PL 12 positivity and another one with OJ positivity. ILD was seen in six of the patients and all of them had NSIP pattern. Mechanic's hand was seen only in one patient. Typical rash of dermatomyositis was seen in five patients. Arthritis was present in four patients and fever in six patients at diagnosis. Only one patient had Raynaud's phenomenon. Eight out of the nine patients had clinical proximal muscle weakness, whereas one patient had subclinical myositis. Bulbar involvement was seen in one patient.
Discussion: In our cohort of patients with Anti Synthetase Syndrome, anti Jo 1 was the most common anti ARS antibody detected, followed by anti PL 7. ILD was seen in 66.6% of patients and all of them had NSIP pattern. All the patients had inflammatory myositis, with one patient having subclinical myositis.
Conclusion: There is much heterogeneity among different patients in the presentation of anti-synthetase syndrome and this variability can be associated with various autoantibodies.
| PC0150: Incidence and predictive factors of restrictive lung disease in rheumatoid arthritis patients | |
Debanjali Chakrabarti, Chiranjit Bal, Kaushik Basu; Departments of Physiology and1Medicine, In-Charge Rheumatology Unit, Medical College and Hospital, Kolkata, West Bengal, India
Background: Rheumatoid Arthritis (RA), a chronic inflammatory disorder of unknown etiology characterised by symmetrical peripheral polyarthritis often resulting in joint damage and physical disability. Several extra-articular manifestations are also associated with RA out of which pleuro-pulmonary involvement is the most important as it bears clinical significance in terms of increased morbidity and mortality. Pulmonary Function Tests (PFTs) are widely used to provide objective measure of lung function for detecting and quantifying such lung diseases.
Objectives: The primary aim of this work is to evaluate and characterize the PFT patterns in RA cases compared to normal controls and find the predictive factors of restrictive lung disease in these cases.
Methodology: Detailed history taking and clinical examination done after informed consent. Pulmonary Function Tests conducted in 50 patients of either sex and above 16 years of age with active RA and also in 50 age and sex matched controls.
Data mining and statistical analysis done.
Results: 38 (76%) out of 50 of cases had evidence of lung disease on the basis of abnormal PFT parameters. The PFT parameters like FVC (%), FEV1(%), FEF25-75% (%), PEFR (L/min) and MVV(L/min) were significantly reduced in cases as compared to controls. 34% of cases had Restrictive lung disease, 4% had obstructive lung disease, 22% had small airway disease and rest 16% had mixed airway disease based on their PFT patterns. Mean duration of disease in RA patients having restrictive lung disease was 4.72 years (SD = 1.2 years) and had high Anti-CCP titers.
Conclusion: Pulmonary Function Tests can be regarded as an effective diagnostic, prognostic and research tool to detect and categorize lung diseases associated with Rheumatoid Arthritis and ultimately guiding treatment protocols.
| PC0151: Osteoporosis in scleroderma: Prevalence, clinical characteristics and correlation with disease phenotype, organ involvement, and vasculopathy | |
Dhaval Tanna, Shounak Ghosh, Lucky Sharma, Wasim Kazi, Shruti Bajad, Rohit Bajaj, Vinay Singal, Rajiva Gupta; Medanta-The Medicity, Gurgaon, Haryana, India
Background: Inflammation is considered to be the causal factor for osteoporosis in rheumatic diseases. However, apart from inflammation, scleroderma is uniquely characterized by vasculopathy, for which clinical features such as digital gangrene and pulmonary arterial hypertension are considered to be manifestations. Studies have shown that in scleroderma patients, digits having severe digital gangrenes have higher prevalence of bone loss in the form of acro-osteolysis. We therefore extrapolate that scleroderma may have higher prevalence of osteoporosis because of systemic vasculopathy apart from inflammation.
Methods: All patients classified as having Scleroderma as per the American College of Rheumatology/European League Against Rheumatism 2013 criteria were included. After written informed consent, demographic profile, clinical features, laboratory and radiology parameters were recorded. Presence of osteoporosis was assessed by two-site dual-energy X-ray absorptiometry (DEXA) scan (hip and lumbar spine) and was correlated with disease phenotype and vasculopathy. The study was approved by the Ethics Committee (MICR- 771/2017).
Results: From June 2017 to May 2019, 115 patients were diagnosed with scleroderma. 90 patients underwent DEXA scan and were included in the present analysis, with 45 healthy controls taken for comparison. Out of the 90 patients, 31 (34.4%) and 3 healthy controls (6.66%) had osteoporosis [p<0.001]. No association was found between osteoporosis and interstitial lung disease, duration of disease and type of serology. In univariate analysis, old age, low BMI, presence of menopause, presence of digital ulcers, PAH, GI involvement and diffuse cutaneous phenotype were significantly associated with presence of osteoporosis (P < 0.05) [Table 1]. No association was found between glucocorticoid intake and prevalence of osteoporosis [Table 2].
 | Table 1: Comparison of clinical and serologic characteristics in patients with and without osteoporosis Click here to view |
 | Table 2: Multivariate analysis was applied with the help of regression models to determine independent predictor of osteoporosis in systemic sclerosis Click here to view |
Conclusion: Scleroderma is associated with an increased prevalence of osteoporosis. Vasculopathy may be an important pathophysiologic mechanism causing bone loss as shown by higher prevalence of digital ulcers, pulmonary arterial hypertension, and diffuse cutaneous phenotype in patients with osteoporosis.
| PC0152: A study of long term disease outcome in patients having rheumatoid arthritis with disease duration greater than 10 years | |
Wasim Kazi, Kaustubh Telang, Lucky Sharma, Dhaval Tanna, Shruti Bajad, Durgarao, Vinay Singhal, Rajiva Gupta; Medanta- The Medicity Hospital, Gurgaon, Haryana, India
Background: Rheumatoid arthritis is associated with multiple extra articular manifestations. Patients with severe disease &/or poor control of disease activity are more likely to develop erosions, deformities and extra-articular manifestations. Studies have shown that most of the manifestations develop within first 5 years of disease. This observational cross sectional study aimed to study different extra articular manifestations in rheumatoid arthritis.
Methods: This cross-sectional study was conducted at Department of Rheumatology and Clinical Immunology at Medanta- The Medicity Hospital, Gurugram from July 2017 till June 2019.
The study subjects were those diagnosed and followed up in the Rheumatology clinic from July 2017 to June 2019. Demographic profile, clinical features, laboratory data, treatment details were noted. Disease characteristics including deformities or other morbidities and associated complications were recorded.
Study was approved by Institutional Research board (IRB). Ethics committee approval was also taken. (Reference number: MICR-789/2017).
Results: A total of 250 patients of Rheumatoid arthritis were studied.Females comprised the majority of the patients included in the study. Median age of onset of RA was 38 years with median duration of 15 years. All patients underwent DAS28 assessment to know the disease activity. Secondary Sjogren's syndrome was observed in 125 patients. Osteoporosis was observed in 53 patients. Interstitial lung disease was diagnosed in 36 patients, nodule in 33, coronary artery disease in 22 and fibromyalgia in 21 patients. Less common extra-articular manifestations observed in our study were peripheral neuropathy, vasculitis, eye manifestations, malignancy and amyloidosis.
 | Table 1: Comparison of our findings with various previous studies Click here to view |
Conclusion: Patients with longer disease duration of more than 10 years were likely to have more extra articular manifestations. Disease duration was found to have direct correlation with these manifestations; Secondary Sjogren's syndrome being the commonest in our cohort followed by osteoporosis and Interstitial lung disease.
| PC0153: Bone density in patients of systemic sclerosis and its relationship with disease related parameters | |
Ankit Patawari, Pradyot Sinhamahapatra, Alakendu Ghosh; Department of Rheumatology, IPGME&R and SSKM Hospital, Kolkata, West Bengal, India
Systemic sclerosis (SSc) is an uncommon connective tissue disease characterized by progressive fibrosis of the skin, vasculopathy, and immune activation. Skeletal manifestations of SSc may include fibrosis of the joint capsule, flexion contractures, thickened tendons, or inflammatory, erosive, and non-erosive arthritis. Recent data suggested an increased risk of osteopenia and osteoporosis in patients with SSc. Risk factors, such as age, low body mass index (BMI), previous fragility fractures, a family history of fractures, use of glucocorticoids, early menopause, systemic inflammatory disease, and active cigarette smoking are classically associated with OP. Before a therapeutic program of research is undertaken, a basic understanding of the epidemiology of low bone density in the setting of SSc is needed, namely the prevalence, determinants of the occurrence of low bone density, and its effect on clinically relevant outcomes.
Objectives:
- Evaluation of Bone Mineral Density in patients with Systemic Sclerosis
- To evaluate the relationship of low Bone Mineral Density in Systemic Sclerosis patients with disease related parameters
Methods: In this Cross-sectional observational study, 100 patients with Systemic Sclerosis who fulfilled the criteria were analyzed from 01 July 2018 - 31 August 2019.
Results: The average disease duration was 9.5 years. BMD showed T score of less than -1.0 in SSc patients. There was low level of Vitamin D levels, and this correlated with the severity of joint involvement, malabsorption syndrome and positivity for certain auto-antibody like anti-Scl 70. There was no correlation with the severity of skin thickening, ILD or pulmonary artery hypertension.
Conclusion: Systemic Sclerosis patients with low BMD have prolonged disease duration, severe joint involvement, malabsorption syndrome and positivity for certain auto-antibody like anti-Scl 70. Patients of Systemic Sclerosis are at a higher risk of losing bone density, especially when other osteoporosis risk factors are present.
| PC0154: Factors predicting mortality in systemic sclerosis – Hospital-based case control study | |
T G Sundaram, Hafis Muhammed, Sakir Ahmed, Durga Prasanna Misra, Ramnath Misra, Amita Aggarwal, Able Lawrence, Vikas Agarwal; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Background: Systemic sclerosis is characterized by high morbidity and mortality due to disease-related or treatment-related complications.
Objectives: We evaluated baseline characteristics of patients of systemic sclerosis who experienced mortality, compared with age and sex matched controls, in a case-control design.
Methods: Records of patients of systemic sclerosis who died between 2000 and august 2019 were reviewed and causes of their deaths determined. We then compared their clinical characteristics and laboratory parameters with age and sex matched live controls in the ratio 1:2.
 | Table 1 Click here to view |
Results: There were a total of 35 deaths. The causes were 12 Interstitial lung disease (ILD) related deaths (34.3%) (11 Lower respiratory tract infections (LRTI), 1 Pulmonary Koch's), 5 Gastrointestinal (GI) sepsis (14.3%), 5 Scleroderma renal crisis (SRC) (14.3%), 3 Cellulitis sepsis (8.6%), 3 severe pulmonary arterial hypertension (PAH) (8.6%), 2 metastatic carcinoma (5.7%), 1 each of myositis with type 2 respiratory failure, viral myocarditis, liver cirrhosis of unknown etiology, 1 disseminated Koch's, 2 unknown sudden deaths probably of cardiac etiology. Median age at death was 49 (Interquartile range, 39-58) years, median disease duration at death was 72 (Interquartile range, 36.75-116) months, and median duration of follow-up was 24 (Interquartile range, 2.5-58.5) months. On univariate analysis. patients who died had greater number of admissions, deranged renal function, higher Aspartate transaminase (AST) levels, lower serum protein and albumin levels. A significantly higher number of patients in the death group were immunosuppressed (58.8%) compared to live controls (41.2%; p= <0.001). There was no significant difference between the two groups in terms of disease manifestations like Modified Rodnan skin score (mRSS), ILD, PAH, arthritis and myositis.
Conclusion: Most deaths in patients of systemic sclerosis were because of infectious complications; more number of hospital admissions, deranged renal functions, higher AST and lower serum albumin at baseline associated with mortality.
| PC0155: Factors influencing the utilization of total knee replacement in osteoarthritis knee: A web-based survey of treating doctors | |
G C Yathish, Yogesh Preet Singh, Lokesh Veerappa1, G. Mallinath1, Hemant Kalyan1; Departments of Rheumatology and1Orthopedics, Manipal Hospital, Bengaluru, Karnataka, India
Background: India, with its aging population is going to face a major healthcare burden due to Osteoarthritis (OA) knee in the coming decades. Total Knee replacement (TKR) which is the most effective way of treating severe OA knee, has wide variation in its utilization. The objective of this survey was to gain insight into the factors that influence the treating doctor in referring a patient to TKR.
Methods: Rheumatologists, Orthopedicians and Physicians were invited to complete a fourteen item web based survey.
Results: Out of the 202 respondents, 140(70%) were Rheumatologists, 47(23.5%) Orthopedicians and 13(6.5%) Physicians. Majority (n=126,63.3%) opined that TKR was underutilized in the treatment of OA knee. Functional demands of the patient were considered as the most important patient factor which influences the decision to refer to TKR by 170 (85.8 %) respondents. Financial status of the patient and the availability of health insurance were considered as important socio economic factors influencing referral to TKR by 100(51.3%) and 70 (35.9%) respondents respectively. High cost of TKR was considered as the biggest barrier for underutilization of TKR by 112(56.6%) respondents. The recent price regulation introduced by governing agencies on knee implants had mixed views with 88(44.2%) saying that it has improved access of TKR to larger population and 84(42.2%) saying it has not done any impact.
Conclusion: High cost and non-affordable population remains the biggest barrier for utilization of TKR in OA knee.
| PC0159: Clinical and angiographic features of adult primary angitis of central nervous system | |
Shyamashis Das, Ashis Datta, A. Shobhana, Sukalyan Purkayastha; Institute of Neurosciences, Kolkata, West Bengal, India
Objective: To observe clinical features, angiographic abnormalities and response to therapy in primary angitis of central nervous system (PACNS).
Methods: Adult patients with PACNS, diagnosed by Calabrese's criteria, were recruited for this longitudinal open label study. All of them had abnormalities in digital subtraction angiography (DSA) highly suggestive of vasculitis (narrowing, occlusion or dilation in a segmental pattern affecting multiple cerebral arteries in absence of changes consistent with atherosclerosis) with raised protein in cerebrospinal fluid. All patients were treated with methyl-prednisolone pulse followed by oral prednisolone and mycophenolate and standard care for stroke.
Results: From November 2016 to May 2019 twenty-three patients (15 females) were recruited. Median age was 45 years (range 32-72 years) and median follow up period was 16 months (range: 3-29 months). Most common clinical feature was headache (86.9%) followed by recurrent stroke (56.5%), speech difficulty (30.4%), loss of consciousness (21.7%), balance problem (17.3%), abnormal sensation (13%) and photophobia (4.3%). Brain MRI showed infarcts in 85.7% and hemorrhage in 14.3%. DSA showed bilateral involvement of vessels in 71.4%, medium vessel involvement in all and both medium and small vessel involvement in 57% patients. Narrowing or occlusion of arterial lumen were multifocal in all. DSA demonstrated typical arterial beading pattern, collateral vessels, sharp cut-off and aneurysm in 52%, 40%, 40% and 4% respectively. Follow-up DSA was done in 8 patients after a median period of 12 months and 3 patients showed definite angiographic improvement and rest did not show any significant changes from initial angiogram. Mean clinical improvement on disability, measured by Rankin disability scale, was 2.5 (range 2-4) from baseline. There was no recurrence or worsening of symptoms in any patient till last follow up.
Conclusion: Combination of mycophenolate and corticosteroid resulted in significant clinical improvement and prevented angiographic worsening in PACNS.
| PC0160: Validation of simplified disease activity score forankylosing spondylitis | |
T G Sundaram, Hafis Muhammed, Amita Aggarwal, Latika Gupta; Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Post-Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Background and Objective: Currently available disease activity measures in Ankylosing spondylitis (AS) are complex and cannot be assessed in a busy out-patient clinic without a calculator. The Simplified Ankylosing Spondylosis Activity Score (SASDAS) was devised to obviate this need. We prospectively validated SASDAS and studied whether juvenile onset (<18 years), early disease (<2 years), and pattern of disease (axial or peripheral) impacted these scores.
Methods: Patients with AS (New York Criteria) were assessed for Bath Ankylosing Spondylitis Disease Activity Index (BASDAI), Ankylosing Spondylitis Disease Activity Score (ASDAS), with ESR and CRP and various AS disease parameters at baseline and at 3 months. SASDAS-ESR and SASDAS-CRP were derived by simple addition of individual components of the ASDAS-ESR and ASDAS-CRP score. PGA31 was defined as active disease. Those with change in ASADAS31.1 were classified as responders. Fisher's r to Z transformation was used to compare correlations.
Results: 107 patients (96 male and 11 female) with median age of 29 years (Interquartile range- 22-38) and median disease duration of 6 years (Interquartile range- 2.5-12) were enrolled of which 84.1% were HLA-B27 positive and 38.3% had juvenile onset. Thirty-four patients were followed-up at 3 months. SASDAS (ESR, CRP) showed high correlation with BASDAI (r=0.85, r=0.82), ASDAS-ESR (r=0.95, r=0.75), ASDAS-CRP (r=0.80, r=0.89); all with p<0.001. The SASDAS CRP but not ESR showed good internal consistency (Cronbach's α 0.68 vs 0.18) while both had discriminative validity and sensitivity to change.
Based on previously proposed cut-offs of disease activity defined for ASDAS-CRP, 4.8, 12.8, 22.8 were optimum cut-offs for classifying inactive, moderate, high and very high disease activity by SASDAS CRP. SASDAS-CRP performed better in late disease (Z=3.04; p=002) and those with adult onset (Z=2.18; p=0.03).
Conclusion: SASDAS is a valid score for measuring disease activity in AS including those with juvenile-onset AS.
| PC0161: Using clinical disease activity index score for assessment of efficacy of abatacept in biological disease-modifying anti-rheumatic drug naïve rheumatoid arthritis | |
A T Atal, S. Kartik; Command Hospital, Pune, Maharashtra, India
Background: The practicability of CDAI score over Disease Activity Score (DAS-28) in assessing disease activity in RA is apparent in Indian OPD setting, obviating the need for acute phase reactant values and complicated calculations. Costimulation blockade with Abatacept in bDMARD naïve patients with Rheumatoid Arthritis refractory to conventional synthetic disease-modifying anti-rheumatic drugs (csDMARDs),is effective.
Objective: To assess the efficacy of Abatacept in bDMARD naïve Indian patients with RA using CDAI score.
Methods: This prospective study included 32 patients with CDAI of >10, who had failed conventional therapy with at least 2 csDMARDs, optimized over a period of at least 1 year. They received Abatacept for 12 months. CDAI was measured at baseline, 3, 6, and 12 months. Primary endpoint was the achievement of Low Disease Activity as defined by a CDAI of ≤ 10.
Results: 32 patients (Males- 05, Females-27) were followed up prospectively. 19 were on triple csDMARDs and remaining 13 on two csDMARDs. 31 patients completed 6-month follow-up: 1(3.1%) was in CDAI remission, 13(40.6%) had low disease activity, 16 (50%) had moderate disease activity and 1(3.1%) had high disease activity. One patient defaulted and was lost to follow up after her 3 month review. The patient with high disease activity underwent a switch to Tocilizumab after 6 months. 30 patients completed 12 months follow up, 8(25%) achieved CDAI Remission while 21(65.6%) had CDAI Low Disease Activity. The remaining 1(3.1%) patient had moderate disease activity. Adverse effects eported during the study period were headache (1 patient) upper respiratory tract infection (1 patient), tinea corporis (1 patient).
Conclusion: CDAI is a convenient tool in assessing response to effective therapy with bDMARD Abatacept.
| PC0162: Efficacy of baricitinib in Indian patients with moderate-to-severe rheumatoid arthritis: Subgroup analysis from RA-BEGIN and RA-BUILD | |
Syamasis Bandyopadhyay, Jyotsna Oak1, Sundeep Upadhyaya2, Anurag Agarwal3, Rohit Arora3, Subhashini Arthanari4; Apollo Gleneagles Hospital, Kolkata, West Bengal,1Kokilaben Dhirubhai Ambani Hospital, Mumbai, Maharashtra,2Indraprastha Apollo Hospital, Delhi,3Eli Lilly and Company, Gurgaon, Haryana, India,4Eli Lilly and Company, Singapore
Background: Baricitinib, an oral selective inhibitor of Janus kinase (JAK) 1 and JAK 2, is approved in more than 50 countries for the treatment of active rheumatoid arthritis (RA) in adults. RA-BEGIN and RA-BUILD studies have shown clinical efficacy of baricitinib in disease-modifying antirheumatic drug [DMARD]-naive patients and in patients with inadequate response to conventional DMARDs.
Objective: To evaluate the efficacy of baricitinib in Indian patients with moderatetosevere active RA.
Methods: This subgroup analysis included Indian patients from 2 international, multicenter, phase 3, randomized controlled trials: RA-BEGIN (N=47) and RABUILD (N=58). In both studies, the primary efficacy outcome was improvement in ACR20 and secondary efficacy outcomes were improvement in ACR50/70, change from baseline in DAS28-hsCRP, change from baseline in HAQ-DI score, SDAI, CDAI, patient's assessment of pain, and patient's global assessment of disease activity. Treatments were compared across all arms including baricitinib 4-mg, methotrexate (MTX), and baricitinib 4mg+MTX in RA-BEGIN and baricitinib 2-mg, baricitinib 4-mg, and placebo in RA-BUILD using logistic regression and analysis of covariance.
Results: Patients who received baricitinib had greater ACR20 response rates at Week 12 in RA-BEGIN (vs MTX) and at Week 24 in RA-BUILD (vs placebo). Similarly, better improvements were observed in ACR50/70 response rates, HAQ-DI, DAS28-hsCRP, CDAI, SDAI, pain, and disease activity in patients who received baricitinib at Week 12 in RA-BEGIN (vs MTX) and at Week 24 in RA-BUILD (vs placebo) [Table 1].
 | Table 1: Efficacy measures up to week 24 in RA-BEGIN and up to week 12 in RA-BUILD Click here to view |
Conclusion: The Results: demonstrated greater improvements in all efficacy outcomes with baricitinib treatment at Week 12 (vs placebo; RA-BUILD) and Week 24 (vs MTX; RA-BEGIN) in Indian patients. These Results: were consistent with those observed in the global population of RA-BEGIN and RA-BUILD studies.
| PC0163: An etiological and clinicopathological study of adult onset nephrotic syndrome in a tertiary care centre of North Bengal | |
Saikat Singh, Debadyuti Mahapatra, Biswadip Ghosh, Pasang L. Sherpa; North Bengal Medical College and Hospital, Siliguri, West Bengal, India
Background: Nephrotic Syndrome describes the clinical condition of heavy proteinuria (>3.0 g/24 h), hypertension, hypercholesterolemia, hypoalbuminemia, edema/anasarca, and microscopic hematuria. It can either be primary(idiopathic) or secondary.
Objective: To study the etiological and clinical and histo-pathological aspects of adult-onset Nephrotic Syndrome and their correlation in a tertiary care centre of North Bengal.
Methods: It is an observational, cross-sectional study. 50 patients were included in the study after meeting the inclusion criterion and taking informed consent. Detailed history was taken followed by clinical examination. After routine and specific investigations were done, patients were sent for USG guided percutaneous renal biopsy and samples were tested using light microscopy and immune-fluorescence study.
Results: Out of 50 patients, 26 were males and 24 were females. Most of them belong to the 25-34 years age group. Most patients were from low socio-economic group. Pedal edema was the most common presenting complaint followed by facial puffiness. According to renal histopathology most common type was FSGS (28%) followed by MGN (22%) and LN (20%). In our study, males dominated in all histologic subtypes except Lupus nephritis, where females predominated. Systemic hypertension was present in 50% of cases. Hematuria was present in 26%. Both the cases of IgAN and MesPGN and all the cases of LN presented with hematuria. 24 hours urine protein excretion or ACR ranged from 3 .5 g to 9 g/ day with average 5.06±1.23. Majority of patients of nephrotic syndrome did not have significant renal dysfunction. Renal dysfunction was seen in 34% of cases but it was 40% among patients of lupus nephritis. Nephrotic syndrome was primary or idiopathic in 80% of cases and secondary in 20% of cases.
Conclusion: FSGS is the leading cause of nephrotic syndrome in adults in North Bengal along with MGN, LN, MCD.
| PC0164: Prospective clinical study in patient with viral illness with arthritis in a tertiary hospital | |
Dharshan Gowda, V P Pandey, D R Ashok Thakur Resident, Professor and Head, Associate Professor
Background: Epidemics of viral fever like chikungunya presenting with fever, rashes and polyarthralgia lasts for 7-10 days. However chikungunya can cause severe chronic arthralgia that can last for months to years. Viral infection show rheumatologic symptoms by immune complex formation and immune dysregulation.
Objectives:
- To study the pattern of fever and arthritis in patients attending MYH OPD
- Follow-up of the patients at 3 months interval for 18 months (from December 2017 to May 2019)
- To evaluate the patients with preexisting morbidity and course of arthritis
- To know prevalence rheumatoid arthritis like condition.
Methods: Observational prospective study involving 262 patients with fever and arthritis presenting to the Maharaja Yashwantrao hospital.
Results: Females 139[53%] affected more than Males 123[47%]
- Age ranging from 18 to 80 years with mean age of 39.68 years. Most of the patients were in the age group of 20-40 years [137 patients; 52.3%].
- Arthralgia 100%, history of fever 53%, fever 47%, rashes 11.8%, headache 27%, family history 7%.
- Joint tenderness 68%, stiffness 35%, movement restriction 40%, edema 22%.
- CRP positive in 148(56.5%), RA factor was positive in 13 (5.0%) patients, Chikungunya IgM antibody was positive in 61 (23.3%) patients, equivocalresult in 5 (1.9%) and Dengue IgM antibodies were positive in 4 (1.5%) patients.
- Joint involvement at the beginning, 3rd month and 18th month are 100%, 80% and 51% respectively.
- EULAR criteria positive in 33 patients in 1st visit, 16 patients in 2nd visit.
- Mean number of joints involved at 1st visit 6.25, 2nd visit 4.05 and 3rd visit 1.9.
- Joints involved-Knee [81%], wrist [78%], small joints [58%], ankle [47%], elbow[52%] and axial[23%].
Conclusion: Chikungunya has high prevalence of persistent rheumatic symptoms. Study shows the pattern of joint involvement, duration of arthralgia in viral arthritis.
 | Figure 1: Comparison of patient having EULAR SCORE more than 6 at different visits Click here to view |
| PC0165: Experience of rituximab in idiopathic inflammatory myositis: Data from Myo-IN registry | |
Ramya Janardana, Aneesa Kapadia1, Sanjiv Amin1, Liza Rajashekhar2, Latika Gupta3, Vineeta Shobha, Ramnath Misra3; Department of Clinical Immunology and Rheumatology, St. John's Medical College Hospital, Bengaluru, Karnataka,1Consultant Rheumatologist, Mumbai, Maharashtra,2Department of Clinical Immunology and Rheumatology, Nizam's Institute of Medical Sciences, Hyderabad, Telangana,3Department of Clinical Immunology, Sanjay Gandhi Post Graduate Institute of Medical Sciences (SGPGIMS), Lucknow, Uttar Pradesh, India
Objectives:
- Study the prescribing pattern and outcome of idiopathic inflammatory myositis (IIM) patients managed with Rituximab
- Study the safety profile of Rituximab
Methodology: Myo-In registry (4 centers) based retrospective data on IIM patients who were given Inj Rituximab in a tertiary care setup over last 5 years. Rituximab was administered either as 500mg or 1g dose 2 weeks apart. All patients were continued on concomitant immunomodulators.
Results: Fifty-five patients of IIM, 42 of whom were females, with a mean (SD) age group of 38(±13.6) years were administered Rituximab. Three patients had juvenile dermatomyositis (JDM), 24 were dermatomyositis (DM), 12 were anti-synthetasesyndrome (ASS), 9 were overlap myositis and 7 were polymyositis [Table 1].
Information on outcome was available at last followup in 40 patients, at 6 months in 37 patients. Median (IQR) duration of follow-up post Rituximab was 24(11,36) months, majority received single course. All patients except one had at least partial improvement by 6 months. Fifteen patients had complete improvement by 6 months and 30 patients had complete improvement by the last follow-up. No significant difference was observed between partial and complete responders at 6 months.
In the subset with available follow-updata (n = 40), infusion reaction was not reported with any of the infusions. Two patients developed serious infection in the form of lower respiratory tract, both survived. No deaths have been reported during the follow-up period.
Conclusion: Rituximab is safe and efficacious in IIM in this cohort. No specific clinical or laboratory factors are associated with early complete response.
| PC0166: Profile of critical illness and its outcome among children with pediatric SLE, admitted in PICU in a tertiary care centre in South India | |
S Indhuumathy Thayammal, T K Shruthi, Mahesh Janarthanan, P S Rajakumar, S Shubha; Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Background: SLE is an autoimmune disease. The outcome of critical illness in adults requiring ICU admission is well established but pediatric counterpart is lacking. Hence we decided to present this study.
Objective: To study the profile of critical illness and its outcome in pediatric SLE.
Methodology: This is a retrospective observational study at PICU, SRMC& RI, SRIHER over 2 months. Pediatric SLE children admitted to PICU with critical illness during Jan 2010 to May 2019 were included. The demographic details, complication and management data were collected from case sheets and studied.
 | Table 1 Click here to view |
Results: Out of 285 admissions among 87 SLE children, 14 developed critical illness needing PICU admissions (16 admissions).The median age at PICU admission was 13yrs (1.5-17), showing a female preponderance 11:3. Average length of PICU stay was 8 days. ARDS(12.5%), pulmonary edema(12.5%), pulmonary thromboembolism(6.25%), pulmonary hemorrhage(6.25%), H1N1 pneumonia(6.25%), Seizures due to CNS vasculitis (18.75%) and cerebral hemorrhage leading to hemiparesis(6.25%) refractory hypertension(12.5%), cardiac tamponade with obstructive shock and severe pulmonary hypertension(6.25%) were the reason for PICU admission. Immunotherapy and supportive therapy given as per unit Protocol. Out of 14 children, 4 succumbed to the complications. Rest 10 are under follow up with medications.
Conclusion: we observed that mortality was highest among younger age group and children presenting late to PICU with complication.
| PC0167: Predictors of early atherosclerosis in children with juvenile idiopathic arthritis | |
Sneha Agarwala, Suparna Guha, Priyankar Pal, S R Pal; VIMS and ICH, Kolkata, West Bengal, India
Introduction: Multiple amenable risk factors affect the initiation of atherosclerosis.Autoimmune diseases like lupus and vasculitis are known risk factors. This study wasundertaken to determine if there is any statistical association between juvenile idiopathic arthritis (JIA) and increased risk of atherosclerosis.
Methods: This is a combined data (serum HDL level, serum cholesterol level and carotid intima-media thickness) of two case control study, conducted independently, comprising of 75 diagnosed cases of JIA, which includes 3 new cases who were diagnosed and followed up since 6 months and rest of the cases were old cases on DMARDs at the time of recruitment, and 50 age and sex matched controls, attending the Pediatric Rheumatology Unit, Institute of Child Health, Kolkata and Vivekananda Institute of Medical Sciences, Kolkata during February 2018 – August 2019.
Results: We found significantly higher serum levels of total cholesterol (p <<< 0.05) and lower serum levels of HDL (p <<< 0.05) as compared to controls. Difference of mean carotid intima-media thickness (CIMT) between cases & controls was significant (p << 0.05). CIMT (mean) of JIA patients was 0.4966. In controls, the mean CIMT (mean) was 0.3774.
Conclusion: Clinical consequences of the atherosclerotic process, in the form of ischemicheart disease, disorders of cerebral circulation, or circulatory disorders of peripheral arteries occur in the adult population, however atherosclerotic changes have their beginning in childhood. With the advent of increasingly effective drugs for treating chronic inflammatory diseases, we may also consider broadening our strategy for early aggressive management of associated co-morbidities. In this study we find convincing evidence for early onset of atherosclerosis in JIA but further follow up studies with extensive population research are needed for validation in pediatric population.
| PC0168: Case series of patients presenting with peripheral ulcerative keratitis at a tertiary care hospital in Western India | |
Prashant Chotalia, Dhaiwatshukla, Rutwiz Mistry, Sapan Pandya, Puja Srivastava; SMT NHL Municipal Medical College and its Affiliated Hospitals, Ahmedabad, Gujarat, India
Objective: To study profile of patients presenting with peripheral ulcerative keratitis (PUKs) to our OPD.
Methods: Patients referred to with Peripheral Ulcerative keratitis (Idiopathic or associated with connective tissue diseases) were included. Their demographic, clinical, serologic al and treatment details were evaluated. Follow up data wherever available was also analyzed.
Results: Three out of 13 were male. Median age was 62 (IQR-40-65). Mean duration of illness was 1.63 ±1.9 years. Demographics and other features in table.
Seven out of 12 were RF, 3 were ANA and 3 were ANCA positive.
Nine patients received Injectable CYC with oral corticosteroids, 2 received Oral Methotrexate and oral corticosteroids. 1 patient received only oral corticosteroids.
Improvement in vision after treatment was recorded in 4 patients, 1 had persistent low vision despite treatment with CYC and Corticosteroids. One patient lost vision in both eyes. Four patients had more than 70% global improvement.
Conclusion: While most cases of PUK were idiopathic, they responded to immunosuppression as it is believed to be an autoimmune manifestation. More follow up data is needed to ascertain which drugs would be best in controlling this disease.
 | Table 1 Click here to view |
| PC0169: Efficacy of Vitamin E in methotrexate induced transaminitis in rheumatoid arthritis: A prospective randomized open-label case-control study | |
Binit Vaidya, Manisha Bhochhibhoya, Shweta Nakarmi; National Center for Rheumatic Diseases, Kathmandu, Nepal
Background: Derange aminotransferases are one of the commonest adverse reaction seen in management of RA with Methotrexate (MTX). Vitamin E was effective in the prevention of hepatotoxicity induced by MTX in the animal.
Objective: To examine the efficacy of vitamin E in MTX induced transaminitis in RA patients.
Methods: A prospective study conducted at a tertiary center for 6 months. RA patients on MTX treatment with deranged aminotransferases <3 fold were included. Patients with previous liver diseases, alcohol intake, muscle diseases, under hepatotoxic drugs and aminotransferases >3 times UNL were excluded. Simple randomization technique used to divide patients with altered LFT treated weekly with oral MTX (7-20mg/week) into treatment (Vitamin E 400mg bid for 3 months) and control group (no vitamin E). Follow up was after 3 months. Statistical analysis done using SPSS. Paired t-test and student t-test were done to compare mean differences. Ethical approval was obtained from Nepal Health Research Center.
Results: Total of 174 cases had increase aminotransferases, 87.5% were female, housewives (69.8%) and the mean BMI was 25.4±4. Out of 174, 80 patients showed deranged aminotransferases under MTX at a dose of 20mg/week. In the treatment group (88), SGPT (IU/L) and SGOT (IU/L) at baseline were 93.8±12.5 and 67.2±8.9 respectively and at follow up 39.4±5.2 and 26±3.5 respectively. There was a significant decrease in the level of aminotransferases (p value < 0.001). In the control group (86), SGPT and SGOT at baseline were 63.0 ± 2.3 and 46.5±1.8 respectively, at follow up 55.7±6.6 and 44.1±4 respectively with p-value >0.569. In subsequent follow-up, 29 patients were given vitamin E, SGPT decreased from 113 to 43.3 and SGOT from 76.3 to 29.3 (p value < 0.05).
Conclusion: Vitamin E significantly attenuates the MTX-induced hepatotoxicity resulting in a decrease in major adverse effect encountered during the treatment of RA.
| PC0170: Clinical profile of neuropsychiatric manifestations in lupus in South India | |
Benzeeta Pinto, C S Sumatha, Ramya Janardana, Kodishala Chanakya, Vineeta Shobha; Department of Clinical Immunology and Rheumatology, St. John's Medical College, Bengaluru, Karnataka, India
Introduction: Neuropsychiatric manifestations (NPSLE) are one of the least understood aspects of SLE. There are very few reports of NPSLE from India.
Objective: To study the prevalence of different NPSLE manifestations and in our cohort and to compare clinical and immunological features of NPSLE with non NPSLE patients.
Methods: This was a retrospective study in a tertiary care referral centre. All patients of SLE diagnosed in our centre for the last 5 years with neuropsychiatric manifestations as per the ACR definitions were included in the study. Consecutive patients of SLE over last 2 years were taken as controls. The clinical and immunological features were compared with controls without NPSLE.
Results: Ninety patients (85 females) with NPSLE and 137 (130 females) consecutive controls were included in the study. All patients fulfilled the SLICC criteria for SLE. Mean age at presentation of NPSLE patients was 29.31±11.84 years [Table 1]. The commonest NPSLE manifestation was seizures followed by cerebrovascular accident. Twenty nine patients had more than one NPSLE manifestation. Antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibody) was tested in 82/90 patients and was positive in 22 (26.83%). APLA positivity was associated with cerebrovascular disease (p 0.021) and cognitive dysfunction (p 0.018). The other NPSLE syndromes were similar in APLA positive and negative patients. As compared to controls, patients with NPSLE had lower prevalence of mucocutaneous and musculoskeletal manifestations. There was no difference found in anti-ribosomal P, lupus anticoagulant and anticardiolipin antibodies between the two groups. Magnetic resonance imaging was available in 45 patients of which 40 were abnormal. Ischemic changes were the commonest followed by nonspecific T2/FLAIR hyperintensities.
 | Table 1: Clinical and immunological features of NPSLE versus control Click here to view |
Conclusion: Seizures and CVA are the commonest NPSLE syndromes in our cohort. The immunological profile was similar in patients with and without NPSLE.
| PM0001: HIV presented with rheumatological diseases | |
Fatma Fayed, May Morsy, Marwa Elkhalifa; Department of Rheumatology and Immunology, Alexandria University, Alexandria, Egypt
Background: Human immunodeficiency virus (HIV) infection is pandemic nowadays, more than 35 million people are infected with HIV, with two-thirds being resident in Africa.[1] The incidence of rheumatic manifestations in HIV infection was reported in about 4 to 71.3% cases in different studies depending on the stage of the disease and musculoskeletal involvements. [2,3]
Objective: The aim of these study to scope the light on HIV associated rheumatic syndromes. Rheumatic disease was a misdiagnosis or not?
Methods: Cross sectional study of patients admitted to rheumatology unit in Alexandria University with a previous diagnosis of autoimmune rheumatic diseases, resistant to treatment, were screened for HIV.
Results: Three patients found to be HIV positive with low CD4+ less than 200 cells mm3 among 130 screened patients. Two patients were diagnosed as Bechet disease due to recurrent oral and genital ulcers. The first one, also had recurrent skin infections associated with bilateral anterior and posterior uveitis. The second one admitted by recurrent oral and genital ulcerations associated with severe oral candidiasis, arthritis, erythema nodosum and positive pathergy test. The third one diagnosed as peripheral spondyloarthritis admitted with low-grade fever, palmoplantar psoriasis as well as acute extensive anterior and posterior uveitis in left eye and chronic anterior and posterior uveitis in right eye with CMV positive.
Conclusion: HIV infection might be misdiagnosed as a rheumatic disease. It is important to screen patients with inflammatory autoimmune rheumatic manifestations for HIV infection for its implications in the diagnosis and management.
| PM0002: Adult onset still's disease with lymphadenopathy mimicking lymphoblastic lymphoma: A case report | |
S Sivansuthan, Narani Aravinthan, M Prieyanka Krishanthi; THJ, Jaffna, Sri Lanka
A 21 years old female patient presented with high grade fever with constitutional symptoms and inflammatory arthritis which last for 4 weeks.
On examination, she was febrile, tachypneic and had features of active, symmetrical, inflammatory arthritis involving large and small joints of hands. Moreover, she had multiple right sided cervical lymphadenopathy.
Her complete blood count showed neutrophilic leukocytosis and anemia (Hb%-8.6g/dl, WBC-20,000/mm3, with-80%neutrophils, 15%lymphocytes). Her inflammatory markers were high (ESR -128 mm/1sthr, CRP 281mg/dl). Her serum ferritin was >1000 and LDH was 1202 U/l. Her Contrast Enhanced Computer Tomography of neck, chest, abdomen and pelvis revealed generalized lymphadenopathy involving right side cervical, bilateral axillary, right side external iliac and inguinal areas. Right cervical lymph node biopsy revealed a high-grade Lymphoma morphology favor for a diagnosis of lymphoblastic lymphoma. However, her Immunohistochemistry study for right cervical lymph node revealed negative CD-20, Nuclear TDT. Bone marrow biopsy revealed no evidence of infiltration and immunohistochemistry study of bone marrow biopsy was not suggestive of Lymphoma. Meanwhile, she was initially treated with non-steroidal anti-inflammatory drugs and has been treated with high dose of systemic steroids later. She responded quickly to steroid treatment and completely resolved within a few days. She has been followed up at rheumatology clinic.
Discussion: Adult Onset Still's Disease is a rare systemic inflammatory condition. Inaddition toclinical features, histological findings of lymph node biopsy may also mimic lymphoma and some authors reported scattered cases leading to confusion with malignant T-cell lymphoma.[3] Immunohistochemistry isimportant in the diagnosis of AOSD in which histologicalfindings, if taken alone, might be misleading.[4]
Conclusion: In a patient with lymphadenopathy, joint involvement and constitutional symptoms eventhe lymph node biopsy shows the features of T-cell lymphoma we need to consider Adult onset ofStills Disease and which should be confirmed with immunohistochemistry.
| PM0003: Rituximab induced lung injury in a young girl with juvenile dermatomyositis: A clinicopathological conference | |
Ankur Kumar Jindal, Mayur Parkhi1, Kirti Gupta1, Gargi Das, Anju Gupta, Amit Rawat, Amanjit Bal1, Dharmagat Bhattarai, Ashim Das1, Surjit Singh Department of Pediatrics, Advanced Pediatrics Centre, Allergy Immunology Unit,1Department of Histopathology, PGIMER, Chandigarh, India
Case Report:
A 13-year-old girl was apparently well till the age of 11 years when she was diagnosed to have juvenile dermatomyositis in view of progressive proximal muscle weakness, heliotrope rash, Gottron papules, elevated muscle enzymes and suggestive magnetic resonance imaging (MRI) changes. She was treated with corticosteroids, intravenous immunoglobulin, intravenous cyclophosphamide, and mycophenolate mofetil. In view of the chronic relapsing course, she was treated with rituximab at 13 years of age. Two weeks after receiving the second dose of rituximab, she presented with a history of cough and fever. At presentation, she was noted to have crepitations in the chest. Laboratory investigations revealed persistent anemia, normal leukocyte counts with lymphopenia and normal platelet counts. She had absent CD20+ B cells on flow cytometry but immunoglobulins (IgG, IgM and IgA) were within normal range. Initial chest X-ray showed a non-homogeneous opacity in the right lower lobe). Subsequent X-rays showed increasing infiltrates in bilateral lung fields and pneumothorax. A clinical possibility of pneumonia with sepsis with multiorgan dysfunction or rituximab induced lung injury was considered. She succumbed to her illness after one month of hospital stay and a partial autopsy was carried out. Histology of lungs revealed changes of diffuse alveolar damage (organizing phase) in the form of hyaline membrane formation, type II pneumocyte hyperplasia, prominent intra-acinar arteries and inter- and intra-alveolar organization. No organism could be identified. The findings were consistent with a diagnosis of rituximab induced lung injury.
Discussion: Rituximab induced lung injury has mostly been reported in adult patients following treatment of malignancies and is much less common in patients with rheumatological disorders.
Conclusion: Rituximab is increasingly being used in various rheumatological disorders and one must always be aware of its side effects and potentially life-threatening complications.
| PM0004: Complete heart block: A rare presentation in rheumatoid arthritis | |
Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM, Medical College, Indore, Madhya Pradesh, India
Rheumatoid arthritis is a chronic inflammatory disease of unknown etiology characterized by inflammatory polyarthritis with systemic involvement. Cardiac manifestations involve pericardium, cardiomyopathy and rarely conduction defects. Here we report a rare presentation of Rheumatoid arthritis as complete heart block which has incidence of <0.1%.
 | Figure 1: ECG suggestive of complete heart block Click here to view |
 | Table 1: Investigation details Click here to view |
Case Report: A 44 year male with no significant past and family history presented with syncope, palpitations and exertional dyspnea of 1-month duration. Pain and swelling of multiple small joints of hands for 5 months.
On examination, pulse rate was 35 beats/minute, BP was 110/70 mm of hg, respiratory rate was 16 cycles/minute. No abnormality detected on cardiovascular and respiratory system examination. Left 2nd MCP and PIP joints were swollen and inflamed. ECG showed complete heart block. Treated with Hydroxychloroquine, methotrexate and anti-inflammatory drugs. Permanent pacemaker was inserted and symptoms improved.
Discussion: Cardiac manifestations in Rheumatoid arthritis include cardiomyopathy, pericardial effusion, valvular involvement, coronary artery disease, and conduction defects. Conduction block manifests as RBBB, hemiblocks or AV blocks of variable degree like Complete heart block which is extremely rare.
Mechanisms involved are 1) granulomatous invasion and fibrosis of conduction system,2) vasculitis of the arterial supply of conduction system,3) hemorrhage into the rheumatoid nodule, 4)inflammatory lesion extension from the aortic or mitral valve, 5) amyloid deposition.
Conclusion: Rheumatoid arthritis involving the conduction system is atypical presentation and manifestation as complete heart block is extremely rare with an incidence of <0.1%. only a few case reports are reported worldwide.
| PM0005: Case of Bechet's syndrome with large longitudinal esophageal ulcers, bilateral sacroiliitis and oral, scrotal and penile ulcers | |
Varghese Koshy, Amit Kumar; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India
Case Report: Patient is a 40-year, male who initially became symptomatic in March 2019 with fever, oral and penile and scrotal ulcers.He also developed dysphagia to both solids and liquids in May 2019.His UGIE revealed 2 large longitudinal ulcers in the esophagus.Biopsy of his scrotal ulcer revealed neutrophilic vasculitis.
MRI of his L-S Spine & SI joints revealed bilateral Sacroiliitis.
Patient showed an excellent response to Inj. Methylprednisolone pulse 1gm OD for 3 days followed by tapering oral corticosteroids and Tab Methotrexate.
There was complete resolution of his oral, penile , scrotal and esophageal ulcers.
Discussion: Esophageal ulcers are uncommon in BD and since their first description by Brodie and Ochsner in 1973, less than 50 cases have been reported worldwide till date.(1) Esophageal lesions include ulcerations, fistulae, strictures and varices. Esophageal ulcerations can be single or multiple and are often associated with ulcerations elsewhere in the GI tract. Rare gastrointestinal manifestations include portal vein thrombosis and Budd Chiari syndrome. (2) Sacroiliitis is an uncommon manifestation (7.5% cases in one study) (3). The presence of both Sacroiliitis and esophageal ulceration in the same patient has possibly never been reported.
Conclusion: Since Bechet's Disease is a clinical diagnosis, a high index of suspicion is a prerequisite to diagnosing the condition and reporting of cases with its varied and myriad manifestations.
References
- Yi SW, Cheon JH, Kim JH, Lee SK, Kim TI, Lee YC, et al. The prevalence and clinical characteristics of esophageal involvement in patients with Behçet's disease: a single center experience in Korea. J Korean Med Sci 2009;24:52-6.
- Bayraktar Y, Ozaslan E, Van Thiel DH. Gastrointestinal manifestations of Behcet's disease. J Clin Gastroenterol 2000;30:144-54.
- Ambrose NL, Haskard DO. Differential diagnosis and management of Behçet syndrome. Nat Rev Rheumatol 2013;9:79-89.
 | Figure 1 Click here to view |
| PM0006: Arthritis in sarcoidosis: Clinical profile and outcome of 11 patients from Nepal | |
Arun Kumar Gupta; Department of Rheumatology, Norvic International Hospital and Arthritis Care Center, Kathmandu, Nepal
Background: Sarcoidosis is a Multi-system Disease of unknown etiology. Ten to 15 of Patients with Sarcoidosis have associated Arthritis. Aim of this Article is to Delineate Articular Manifestation & Clinical Profile of Sarcoidosis from Nepal.
Methods: All Cases Records of 11 Adult Patients with Sarcoidosis between 2011 & 2019 were Prospectively Reviewed. Joint Involvement was assessed Clinically, Classified as Acute or Chronic respectively. All Patients were Follow Up every 2-3 weeks and Monitored Joints Involvement and Clinical Profiles. Their Treatment and Outcome were Recorded.
Results: Joints Involvement at the Time of Diagnosis was 81.81%. The pattern of Joint Involvement Revealed the Ankle and Wrist to be the most Commonly Affected in Both the Groups. Shoulder, Elbow, Metacarpophalangeal, Proximal Interphalangeal Joints of Hands and Knee Joint Involvement were Significantly more Common in Chronic Sarcoid Arthritis. Other Clinical Features were Fever 83%, Uveitis 36.36%, Peripheral Lymphadenopathy 54.54%, Difficulty in Breathing 45.45%, Skin Lesion (Erythema Nodosum) 54.54%, Hepatosplenomegaly, Weight Loss and Arthralgia. Imaging Findings included: Hilar Adenopathy 36.36%, Pulmonary Infiltrate 45.45%.
Four out of 11 Patients with Acute Oligoarthritis followed over a Median of 2.5 Years had achieved complete Remission. Five patients of Chronic Sarcoid Arthritis associated with Pulmonary Infiltration and Skin Lesion had been Treated with Steroid, Methotrexate and Hydroxychloroquine Respectively. All Patients has assessed by Resolution of Clinical Symptoms & Improvement in Erythrocyte Sedimentation Rate, C-Reactive Protein, Angiotensin-Converting Enzyme Levels and Spirometry Parameters.
Conclusion: Adult Sarcoidosis with Predominant Arthritis seems to Respond well to Systemic Steroid &Methotrexate. The Ankle Joints being the Most Commonly affected in Acute Oligoarthritis Patterns. Knee & Shoulder Joint Involvement were more common in Chronic Sarcoid Arthritis. Uveitis, Peripheral Lymphadenopathy and Skin Lesion were more common in Both Acute and Chronic Sarcoid Arthritis. Delay Diagnosis, Ocular and Pulmonary Involvement are Probably associated with Poor Outcome.
| PM0007: Idiopathic C1 esterase inhibitor deficiency: A rare cause of acquired angioedema | |
Sanjana R Badami, Sudhir Mehta, R K Bhimwal; SMS Medical College, Jaipur, Rajasthan, India
C1 esterase inhibitor deficiency (C1INH-AAE) is a rare self-limited disease of recurrent episodes of angioedema without urticaria. Idiopathic cause accounts to less than 10 percent of C1INH-AAE. A forty-year-old male presented with self-limiting recurrent episodes sudden onset of facial puffiness, difficulty in breathing and pain abdomen occurring at an interval of three to four months for last 1.5 years without any precipitating etiology lasting for 48 to 72 hours. There was no history of fever, rashes, pruritus, personal or family history for allergic disorders and no exposure to environmental allergens or any drug allergy. Routine investigations were negative. Lymphoproliferative disorders, B cell malignancies, autoimmune disorders were ruled out. Low serum complement C4 levels, c1q and C1INH levels established the diagnosis of C1 Esterase inhibitor deficiency. No treatment was initiated as symptoms recovered spontaneously. C1 INH- AAE should be considered in a patient who presents with isolated angioedema (without urticaria) in the fourth decade of life or later without family history of angioedema. All patients with acquired angioedema should be evaluated for an underlying B cell lymphoproliferative disorder at the time of diagnosis and repeated annually.
 | Figure 1 Click here to view |
| PM0008: A tetrad of fever, pain abdomen, diarrhea and anasarca- unraveling the enigma | |
Arka Bairagya, Kaushik Basu, Anupam Sarkar, Debarup Das; Medical College and Hospital, Kolkata, West Bengal, India
Case Report: 31 years old female presented with fever, loose stools and abdominal pain for 1 month along with gradually progressive bilateral pedal swelling followed by abdominal swelling for 3 weeks. Fever was associated with myalgia, arthralgia and colicky abdominal pain; poorly localized and non-radiating. She also complained of vomiting and loose stools, 3-4 times per day. Stool contained only mucus and no blood. On examination there were mild pallor, bipedal pitting edema, moderate ascites and left sided pleural effusion. Investigations revealed anemia, moderate proteinuria, hypoalbuminemia, high fecal calprotectin and normal CRP. Infective etiologies ruled out from ascitic fluid study, urine culture, blood culture and hemogram. CECT abdomen showed small intestinal wall (jejunal) edema, thickening, target sign and comb sign. UGI endoscopy: nonspecific duodenitis. Colonoscopy: nothing significant. ANA (hep2) 4+ coarse speckled (1:160), 3+ anti Sm, low C3 and C4, high anti ds-DNA. Other causes of chronic diarrhea were ruled out. Finally a diagnosis of lupus enteritis was made. Inj. methylprednisolone 1 gm i.v. was given for 3 days followed by tab prednisolone 1mg/kg/day and inj. Cyclophosphamide 1gm i.v. The patient started gradually improving after 1 week.
Discussion: Lupus enteritis is a rare complication of SLE. Patients with SLE can present any time before or after diagnosis of SLE. If diagnosis is delayed intestinal necrosis and perforation can occur. One should rule out other common and infective causes of pain abdomen first. No CT feature is specific for lupus enteritis. Biopsy is usually not required for confirmation but can be done to exclude other possibilities. Intravenous steroid is an effective therapy.
Conclusion: Lupus enteritis is a poorly known cause of abdominal pain in SLE patients with distinct clinical features and therapeutic outcome. High index of suspicion is required to make a timely diagnosis in order to prevent further complications.
| PM0009: Not so uncommon cause of nephropathy in a case of Spondyloarthropathy | |
Paras Kathuria; VMMC and Safdarjung Hospital, New Delhi, India
Case Report: I am presenting a case of a 35-year-old male who presented to us with complaints of low backache for 3 months which used to get increased on resting along with early morning stiffness. His inflammatory markers were raised. On further evaluation he was found to be having bilateral sacroilitis with HLA B27 positivity and a diagnosis of spondyloarthropathy was made. On reviewing old records patient had history of reduced urine output along and pedal edema 1 year back with deranged KFT (62/1.5). Urine examination revealed significant proteinuria and hematuria with active sediments. Kidney biopsy showed IgA nephropathy. He was managed with ACE Inhibitors and steroids to which he responded. His KFT and urine examination were normal when he presented to us. So a diagnosis of Spondyloarthropathy with Ig A nephropathy was made and patient was started on NSAIDs to which he partially responded, we could not start TNF inhibitors in this patient due to financial constraints.
Discussion: Renal abnormalities in ankylosing spondylitis is seen in approximately 5-8% of cases, with etiologies such as amyloidosis being most common followed by drug induced. IgA nephropathy being the third most common cause with prevalence ranging from 20-30% of all causes of renal abnormalities. Most of the cases described in literature were diagnosed in established case of ankylosing spondylitis, however in this case Ig A nephropathy preceded the onset of ankylosing spondylitis which is very rare.
Conclusion:
- Renal evaluation including urinalysis should be done in all cases of spondyloarthropathyand if found abnormal should be worked up for the cause.
- Ig A nephropathy is not an uncommon cause of nephropathy in rheumatological disorder and if present should be appropriately managed.
- Ig A nephropathy may even be present before the onset of spondyloarthropathy.
| PM00010: Adult onset still disease – An uncommon etiology of nonresolving pneumonia: A rare case entity | |
Anil Kumar Behera, Sarat C V Talluri, K Bchetanreddy; Care Hospital, Banjara Hills, Hyderabad, Telangana, India
Introduction: Adult onset stills disease is a rare systemic inflammatory disorder of unknown etiology and pathogenesis with high spiking fever accompanied by several systemic manifestation. A few reports exist in medical literatureexplaining pulmonary manifestation of(AOSD).one rare case entity of(AOSD) with non-resolving pneumonia is being reported.
Case Report: A 33-year-old male, known case of pulmonary tuberculosis and TB lymphadenitis presented with high spiking fever, dry cough, dyspnea and pleuritic chest pain. On examination, patient was tachypneic, hypoxic, with bilateral basal crepitations.hemogram showed leukocytosis, high erythrocyte sedimentation rate and c- reactive protein. Chest imaging showed bilateral mild pleural effusion with mediastinal lymph nodes. Pleural fluid analysis showed transudate, sputum culture negative and other laboratory investigation. Procalcitonin and autoimmune workup was negative. Suspecting stills disease, serum ferritin was very high. Patientdeveloped polyarthritis and transaminitis. Patient did notrespond to any antibiotics. Patient was diagnosed to have AOSD using yamaguchicriteria. Patient was started with naproxen, hydroxychloroquine and IV steroids. Patient improved symptomatically and followed up with oral steroids.
Conclusion: AOSD is an uncommon immunological disorder of unknown etiology with recognized increased frequency, diagnosed by yamaguchicriteria. Infectious diseases, malignancy and rheumatic disorders must be kept in mind as an exclusion for those patients. In our patient, diagnosis of AOSD was made using yamaguchi criteria with following clinical and laboratory values like spiking fever, arthralgia, leukocytosis and elevated levels of aminotransferase and high serum ferritin.
| PM0011: Antineutrophil cytoplasmic antibody-associated vasculitis and systemic lupus erythematosus overlap syndrome: Case report | |
Lapsiwala Mehul, Patel Krunal, Goel Anshul1; Shalby Multi-Specialty Hospital, Surat, Gujarat,1Vivekanand Medical Institute, Palampur, Himachal Pradesh, India
Case Report: We report case of 50yrs(F), presented with fever, arthritis, maculo-papular rash, photo sensitive rash, hematuria, cough. On evaluation, ANA-1:320 (homogenous), anti-dsDNA++, S.Creatinine-2.3 mg7dL, CRP-110mg/L, B/L non-homogenous opacities in lung-fields on X-ray, Urine routine: Proteinuria 3+, >100 RBCs/hpf and casts, protein-creatinine ratio 3.97, ↓S.C3(60mg/dl), c-ANCA-1:40, MPO-positive(>200U/ml). CT-chest: B/L sub-centimetric nodules, tree-in-bud appearance, scattered consolidation patches, multiple enlarged hilar, mediastinal LNs. Kidney biopsy: focal necrotizing glomerulonephritis, cellular crescents and glomerular thrombosis, interstitial inflammation without immunofluorescent deposits. The diagnosis of SLE with AAV was based on clinical, serological, histo-pathological and CT scan abnormalities according to the ACR criteria. She received non-invasive ventilation, 3-pulses prednisolone and supportive therapy. Once stabilized, she received cyclophosphamide 1000mg monthly for 6 months with tapering prednisolone dose. Follow-up 6 months CT-chest showed resolved abnormalities, urinalysis and urine protein/creatinine ratio-negative for proteinuria/hematuria. She is asymptomatic for 9-months.
Discussion: SLE and AAV share clinical features, like arthritis, cutaneous lesions, renal involvement. Patients meeting classification criteria for both SLE/AAV are categorized as SLE-AAV overlap syndrome. They are predominantly women. In these cases, renal histopathology distinguishes AAV from SLE vasculitis. ANCA prevalence is 31% in lupus patients and vasculitis prevalence in SLE is 11-36%. Lupus nephritis is immune complex-mediated glomerulonephritis. Whereas, renal involvement in AAV is pauci-immune crescentic glomerulonephritis. However, there are LN patients (class-IV-segmental, class-III) who develop extensive segmental necrosis, crescent formation without subendothelial immune deposit but have positive P-ANCA+MPO antibodies. They resemble AAV glomerulonephritis. In SLE/AAV syndrome reported so far, renal pathology is classified either LN or pauci-immune GN according to the predominant type lesion. Majority (75–80%) have mesangial deposits and interstitial inflammation. Our patient had pure AAV nephritis.
Conclusion: This case suggests, SLE-AAV overlap syndrome should be considered in differential diagnosis of subjects with LN or AAV, renal biopsy should be considered in any AAV or LN patients.
 | Figure 1 Click here to view |
| PM0012: Case of SLE presenting as SJS-TEN overlap | |
Varghese Koshy, Asmita Sinha, Rahul Rai, Aditya Bakshi, Pooja Devi; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India
Patient is a 62-year, female, who initially became symptomatic in April 2019 with fever, and loss of appetite. There is history of consuming alternative systems of Medicine, temporally, following which she developed extensive generalized maculopapular rash with oral mucosal ulcerations and subsequently bullae formation and degloving of skin. She was initially considered to be a a case of SJS-TEN overlap with DILI (Drug induced Liver injury) and Pancytopenia and polyserositis.
She was managed on the 1st admission in April/May 2019 with IVIg followed by oral corticosteroids. She recovered well. Her Serology was suggestive of SLE ( ANA by IIF +ve Homogenous, Anti dsDNA +ve, Anti Sm +ve, Anti Nucleosome +++, Anti Histone +ve, SSA +ve) with decreased C4 level.She developed a recurrence of SJS-TEN overlap in June 2019 and was then managed with Pulse dose of Inj Methylprednisolone 1gm OD x 3 days and was also started on Tab MMF. She was administered 2 doses of Inj Rituximab 15 days apart.
Discussion: Although SJS and TEN are almost invariably due to medications, they may, rarely, be an initial presentation of lupus.
Conclusion: SLE presenting as SJS-TEN overlap is rare and the management of such a patient is challenging requiring a team approach and b decision making.
 | Figure 1 Click here to view |
References
- Lee HY. SLE presenting as SJS & TEN: A report of 3 cases. Lupus 2011;20:647-52.
| PM0013: H syndrome: A novel genodermatosis mimicking IgG4 related disease | |
Benzeeta Pinto, Gautham Arunachal1, Rahul Sethia2, Ashish Aggarwal2, Vineet Ahuja2; Department of Clinical Immunology and Rheumatology, St. John's National Academy of Health Sciences,1Department of Human Genetics, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka,2Department of Gastroenterology, All India Institute of Medical Sciences, New Delhi, India
Case Report: 24-year-old male was symptomatic for the last five years with abdominal pain and loose stools alternating with constipation. He noticed thickening and darkening of skin on both his lower limbs and dryness of mouth and eyes with caries of multiple teeth. Hyperpigmentation and induration was noted on both lower limbs extending till lower abdomen prominent on inner thighs and sparing both the knees [Figure 1]. A firm mass was noted in the pelvic region. Colonoscopy revealed superficial ulcers with mild luminal narrowing in the distal ascending colon with dense inflammatory infiltrate on biopsy. PET CT revealed FDG avid multiple parenchymal opacities in both upper lobes and sheet like soft tissue thickening with mild FDG uptake involving thoracic aorta, retroperitoneum and pelvic fascia with skin and subcutaneous involvement in inguinal and thigh regions. FDG uptake was also noted in the hepatic flexure with thickening of the wall. Double inferior vena cava was seen. Biopsy of pelvic mass revealed extensive fibrosis. Immunostaining for IgG4 was negative. IgG4 levels were within normal limits. A diagnosis of H syndrome was considered in view of characteristic cutaneous findings. Clinical exome sequencing revealed a previously reported pathogenic variant c.1330G>T [p. E444Ter; HGMD ID- CM093097] in homozygous state in SLC29A3 confirming the clinical diagnosis of H syndrome.1 He is started on tocilizumab with symptomatic response.
 | Figure 1 Click here to view |
Discussion: H syndrome is a rare autosomal recessive disease characterized by cutaneous hyperpigmentation, induration and hypertrichosis along with numerous systemic manifestations The clinical manifestations are heterogenous and include short stature, diabetes mellitus, hypogonadism, flexion contractures and heart anomalies. Only 10 cases are reported from India.
Conclusion: Tocilizumab may be an effective treatment for H syndrome.
| PM0014: An unusual case of gouty arthritis in a young woman with pregnancy | |
Lubna Khurshid, Suraj Chaudhary, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, New Delhi, India
Case Report: A 39-year-old woman presented with acute inflammatory arthritis of the right knee in her 8TH week of pregnancy. She had a history of episodic paraparesis 20 years ago. Initially treated as polymyositis with high dose of steroids. A few months later she was correctly diagnosed as hypokalemic periodic paralysis and treated with fluids and potassium supplements. 3 years later she developed episodic intermittent pain and swelling involving both knees and both the shoulders. She was diagnosed as inflammatory polyarthritis and treated with DMARDs with some relief. However she had a flare of right knee arthritis in March 2019. Synovial fluid analysis showed monosodium urate (MSU) crystals. Serum uric acid (SUA) was 9.8mg/dL. She was managed with allopurinol, colchicine and glucocorticoid (GC). She improved but had another flare of arthritis during pregnancy. Allopurinol was discontinued and she was maintained only on colchicine and GC.
Investigations: ESR 109 mm 1st hr, Hb-9.8 gm/dL, WBC 15000/cmm, platelets 3, 85, 000/cmm. KFT and LFT were normal, SUA 9.8mg/dL, Synovial fluid from the right knee joint showed negatively birefringent MSU crystals under polarized light microscopy. Test for the enzyme HPRT and PRPP was not available; could not be done.
Discussion: Gout is rarely diagnosed in young, premenopausal women. Its protective effect is attributed to estrogen therefore if found in a young woman, secondary causes of hyperuricemia should be investigated (Table 1). Diagnosis is based upon clinical findings and demonstration of MSU crystals in synovial fluid. Treatment should be targeted to keep SUA < 5 mg/dL.
Conclusion: Asymmetrical seronegative polyarthritis with acute intermittent attacks should be considered to be due to gout even in persons in young age group and carefully investigated as it is a treatable condition. Due to its rarity in young woman its treatment in pregnancy remains a challenge.
| PM0015: An elderly patient of Takayasu Arteritis presented with hypertensive heart failure | |
Bharat Kumar, Rohit Mathur, Sonu Pandit, S Veena, N I Galib Mirza, Mahendra Kumar Garg; All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
Introduction: Takayasu arteritis or pulse-less disease is a rare chronic inflammatory large vessel vasculitis involves primarily aorta and its branches; affecting the females in the second and third decade. The main pathological changes in vessel wall are thickening, fibrosis, thrombosis, stenosis, formation of aneurysm and these changes are due to cellular immunity against the endothelial cell. Manifestations range from asymptomatic pulseless patient to catastrophic neurological or renal involvement. Lack of specific treatment and delayed diagnosis causes high morbidity and mortality of the disease.
 | Figure 1 Click here to view |
Case Report: A 59 years old female from Barmer, Rajasthan came to emergency with acute shortness of breath of 2 hours duration. She also complained of headache, decreased vision and decreased urine output for 7 days. On examination, the patient was conscious, disoriented and had findings suggestive of heart failure. Patient left radial &brachial pulse was absent and had blood pressure of 240/160 mmHg which was treated as hypertensive emergency with NTG and labetalol infusion in A & E. Patient was further investigated. MRI brain with angiography shows posterior reversible encephalopathy syndrome (PRES), complete occlusion of left external carotid and 3rd part of subclavian artery and saccular pseudo-aneurysm of left ICA. Renal Doppler suggests bilateral renal artery stenosis. Fundoscopy suggestive of grade 2 Retinopathy (Takayasu retinopathy). All the findings were consistent with Takayasu arteritis complicated by renal, neurological, eye and cardiac involvement. Steroids were started and blood pressure was maintained with antihypertensive. Despite optimal treatment, the patient had progressive renal failure which leads to mortality.
Conclusion: Takayasu arteritis is rare in the elderly but it can be considered in a female with malaise, fever, pain abdomen, and hypertension with asymmetrical or absent pulses. Though it rapidly involves multiple organs and can be devastating but early diagnosis and prompt treatment with steroids can prevent further complications and mortality.
| PM0016: A patient with swollen hands | |
Lubna Khurshid, Suraj Chaudhary, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, New Delhi, India
Case Report: 75 years man presented with Swelling of both the hands x 4 months. He was being managed as carpel tunnel syndrome by an orthopaedician without relief.On examination the hands were grossly swollen (dorsal and planter aspect) [Figure 1]. Additionally, there was marked restriction of cervical, dorsal and lumbar spinal movement in all planes. There was past history of CAD, episodic monoarthritis treated as gouty arthritis intermittently, and tubercular lymphadenopathy – taken ATT for 2 years 18 years ago.
 | Figure 1: Swelling of hands Click here to view |
Investigations: ESR 33 mm 1st hr., Normal hemogram. The X Ray of the cervical and dorsolumbar spine showed loss of lumbar lordosis, marked syndesmophytes and grade 3 sacroiliitis respectively [Figure 2]. HLA B27 test was negative. USG hands suggested synovial hypertrophy at radiocarpal joints with chronic tenosynovitis and subcutaneous tissue hypertrophy of bilateral hands and wrists.
He was managed as a case with axial and peripheral SpA associated with CAD, treated with methotrexate, sulphasalazine, and NSAIDs. If he does not respond, TNFi may be considered.
Discussion: SpA as a group are a common rheumatic disease with a prevalence of 0.5-1.9% making them at least as common as rheumatoid arthritis. SpA is a condition with broad spectrum of clinical manifestations, laboratory abnormalities and imaging features. As a result, it is difficult to recognize all of its protean manifestations in clinical practice. The pessimistic scenario of a late case has changed dramatically since the advent of TNFi drugs.The early and correct diagnosis, although still remains a challenge, is important with the availability of a number of highly effective drugs.
 | Figure 2: Radigraphs showing Grade 4 sacriiliitis and lumbar lordosis, syndesmopytes and possible compression fracture with anterior wedging of L1 vertebra Click here to view |
| PM0017: Monoarthritis with skin rash in a middle aged male: An unusual case | |
Suraj Chaudhari, Lubna Khurshid, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspeciality Hospital, Vasant Kunj, New Delhi, India
A 45 year old man with 6 years of T2DM presented with mildly itchy rash over the trunk and scattered areas over arms. The lesions were erythematous, well defined, slightly scaly papules and plaques, with hypopigmented borders in some lesions. He had swelling below his ears and on the lateral side of his neck since 2018. There was right elbow swelling, pain worst in last 3-4 days treated with aspiration and IAS in another hospital, without relief. He consulted a dermatologist; a skin biopsy was performed (reported as sarcoidosis). He had bilateral parotid and cervical lymph node enlargement and broad nodular nose. The examination of the other systems was unremarkable.
Investigations:
ESR 50 mm 1sthr, CRP 156, Hb10.70gm/dl, WBC 5500 /cmm, Platelets18800/cmm, ACE levels 60 u/l, USG abdomen - hepatosplenomegaly & retroperitoneal lymphadenopathy. CECT chest – lymphadenopathy without necrosis suggesting sarcoidosis.
Final Diagnosis: Extra-pulmonary sarcoidosis with skin lesions and right elbow monoarthritis.
Treatment prescribed: methotrexate 20 mg weekly, hydroxychloroquine 400 mg/day and methylprednisolone 4 mg daily.
 | Figure 1: Before treatment (a) Lupus perino (b) Trunk of the patient erythematousslightly scaly papules and plaques over the trunk. After treatment (a) A significant decrease in lupus perino (b) A significant decrease in plaques and papules over the trunk Click here to view |
Follow-up: after 4 weeks of treatment joint symptoms had disappeared with marked clearing of the skin lesions.
Discussion: Sarcoidosis is a chronic multisystem disease of unknown etiology mostly involving lungs, but frequently shows extra-pulmonary manifestations that might be difficult to recognize and treat.
Cutaneous lesion includes erythema nodosum, subcutaneous nodules, maculopapular eruptions, lupus Perino. In MSK involvement any type of arthritis may occur though monoarthritic, as seen in our patient, is uncommon. Acute form of sarcoidosis with bilateral hilar lymphadenopathy and ankle periarthritis with EN is called Lofgren's syndrome.
Conclusion: In the absence of any RCTs for treating sarcoidosis, treatment is based upon the experience of the clinicians who have treated large number of patients. Glucocorticoids and hydroxychloroquine with methotrexate and TNF-alpha inhibitors as steroid-sparing agents are used with satisfactory Results:
| PM0018: Nocardia brain abscess mimicking tuberculoma in a systemic lupus erythematosus patient | |
Mukta Wyawahare, M Nidhish Chandra; Department of Medicine, JIPMER, Puducherry, India
Background: Patients on immunosuppression for Systemic Lupus Erythematosus (SLE) are prone for atypical infections. Here we present a case of Nocardia farcinia brain abscess in an SLE patient on immunosupression who presented with altered sensorium.
Case Details: A 41-yearfemale, housewife,a known case of Systemic Lupus Erythematosus (SLE) on Prednisolone 30 mg once daily, Hydroxychloroquine 200 mg once daily, Methotrexate 10 mg weekly for the past 8 months now presented to us with altered sensorium.Initial MRI showed Multifocal areas of T2 iso to mild hypointense signals in right cerebellum, left temporal periventricular region, right occipital lobe that showed diffusion restriction with ring enhancement. Mild perifocal edema and diffuse leptomeningeal enhancement were also present. MRS showed elevated lactate peak and choline to creatinine ratio>1 suggestive of tuberculosis. Her sensorium worsened despite starting ATT and an external ventricular drain was placed in view of hydrocephalus. CSF-gram stain revealed gram positive, filamentous organism and subculture grew Nocardia farcinia identified by MALDI TOF.Brain heart infusion blood agar showed buff coloured dry, cerebriform colonies. and Modified Ziehl Nielsen filamentous, branching staining demonstrated thin, partially acid-fast bacteria in a Background of many polymorphonuclear leukocytes suggestive of Nocardia. After nocardia was isolated in culture, intravenous trimethoprim-sulfamethoxazole (15mg/kg of TMP divided in three doses), intravenousamikacin (7.5mg/kg every 12 hours) and intravenous meropenem (500 mg every 6 hours) was started. Later patient developed Extraventricular drain related shunt infection during which Acinetobacter was isolated from CSF. Patient developed external ventricular drain infection and septic shock and expired after 2 months of treatment.
Discussion and Conclusion: In an immunocompromised patient presenting with multiple ring enhancing lesion in the brain, we have to think of atypical infections like nocardia as differentials especially when patient is not responding to routine treatment.
| PM0019: A rare case of Behcet's disease misdiagnosed as rheumatoid arthritis/Hansen's disease | |
BaikunthaPanigrahi, Bidyut Kumar Das, Saumya Ranjan Tripathy, Manoj Kumar Parida; S.C.B.Medical College, Cuttack, Odisha, India
Case Report: A 38-year old male was on treatment (methotrexate and sulfasalazine) for RA since 2008. In 2012, he developed episode of choroiditis and left forearm panniculitis (biopsy-proven) which was managed with systemic steroids. Since 2016, he has suffered from recurrent, multiple ulcers over bilateral lower limbs. In 2018, he developed bilateral ulnar claw hand. Although, split skin smear was negative, he was started on MDT for Hansen's by local physician based on tender and thick bilateral ulnar nerves. He was referred to Rheumatology for further evaluation. On examination, he had non-healing ulcers over dorsum of left foot, right 1st, 2nd and 5th toes. Bilateral wrists were tender and swollen. There was a superficial tortuous vein over abdomen with flow downward from epigastrium to umbilicus. There was a dermatomal loss of sensation along left sural nerve, bilateral ulnar claw hands. CBC, RFT, LFT, and urine R/M were normal. ESR:69 mm 1st hour, and CRP:17mg/l were raised. RF, ACPA, ANA and ANCA were negative. NCS: sensory motor axonal polyneuropathy. Doppler ultrasound revealed left sapheno-femoral incompetence. Biopsy of ulcer-margin revealed leucocytoclastic-vasculitis. Sural nerve-biopsy revealed vasculitis and was AFB-negative. CT-angiography revealed right brachiocephalic and SVC thrombosis. APLA was negative, Factor V Leiden mutation absent and serum homocysteine was normal. Pathergy test was negative. However, HLA-B51 was positive. He was started on oral prednisolone (1mg/kg) and azathioprine and has shown marked improvement.
Discussion: The patient had arthritis, vasculitic ulcer, neuropathy and venous thrombosis with history of choroiditis [score=4 as per ICBD-criteria, 2014]. The diagnosis of RA-vasculitis is unlikely, as RF and ACPA are negative. Nerve biopsy was AFB-negative and venous thrombosis does not occur in Hansen's. Although Pathergy is negative, HLA-B51 supports diagnosis of Behcet's disease.
Conclusion: Presence of venous thrombosis is an important clue to diagnosis of Behcet's in a setting of vasculitis.
 | Figure 1 Click here to view |
| PM0020: Multifocal cutaneous tubercular abscess in a SLE patient presenting as MAS | |
Siddhartha Satapathy, Lavina Patnaik, Rina Tripathy1, Manoj K Parida, Saumya R Tripathy, Bidyut K Das; SCB Medical College,1SVPPGIP, Cuttack, Odisha, India
Case Report: A 18 year old female who was diagnosed case of SLE nephritis mucocutaneous for 1.5 years with history of MAS on corticosteroid presented with fever and multiple swelling over right elbow, medial and lateral aspect of foot. The swellings were tender, fluctuant with purulent discharge.
Laboratory investigations revealed normal complete blood count with elevated ESR and CRP and low C4, normal LFT and RFT.FNAC of the nodule showed inflammatory lesion. Chest radiograph was normal. Pus from the site of lesion showed acid fast bacilli and MTB was detected on CBNAAT with sensitivity to rifampicin. Based on these observations, a diagnosis of multifocal tubercular abscess was made in this SLE patient and cat I ATT was started and is on follow up.
Discussion: There is a high prevalence of tuberculosis in SLE which can be attributed to multiple immune abnormalities and related to immunosuppressive therapy. Extrapulmonary involvement is more frequent than pulmonary in SLE.
Conclusion: The possibility of tuberculosis must be kept in mind in case of subcutaneous abscess and nodules non responsive to antibiotic therapy and drainage. Awareness of unusual presentation of tuberculosis is important for early diagnosis and proper management.
| PM0021: An unusual presentation of multifocal musculoskeletal tuberculosis | |
Lavina Patnaik, Siddhartha Satapathy, Manoj K Parida, Saumya R Tripathy, Bidyut K Das; SCB Medical College, Cuttack, Odisha, India
Case Report: A 35-year-old man presented with 6 months history of swelling of right wrist and left ankle, and 3 months history of pain over thoracolumbar spine without any significant accompanying symptoms of fever, weight loss, loss of appetite or night sweats. Examination revealed kyphoscoliosis, swelling over left ankle and right wrist, hyperreflexia with bilateral flexor plantar response and no sensory or motor deficit. Laboratory reports revealed normal complete blood count with high ESR and CRP, elevated urea (51mg/dl) and creatinine (2.4mg/dl) and normal LFT; chest radiograph showed homogenous opacity adjacent to right hilum and HRCT thorax showed multiple paravertebral abscess. Ultrasonography of abdomen and pelvis showed features of chronic medical renal disease. MRI of right wrist and left ankle showed features of chronic inflammatory arthropathy possibly due to Koch's and MRI spine revealed disseminated spinal tuberculosis with bony erosions at C6, C7, D4, D5, D6, D7, D8, D9, D10, L5 &S1 and paravertebral abscess extending from C6-D12 and L5-S1. Synovial fluid examination from left ankle showed acid fast bacilli. HIV ELISA was non-reactive. Based on these observations, a diagnosis of multifocal skeletal tuberculosis was established and patient was started on Cat I anti-TB chemotherapy and is on follow-up.
Discussion: Extrapulmonary tuberculosis accounts for 15%-20% of all cases of tuberculosis, with skeletal tuberculosis comprising 15% cases of extrapulmonary TB.
The present study is rarest of rare case of non-contiguous multifocal osteoarticular tuberculosis in a healthy 35-year-old male with negative HIV status which should be considered in patients with long standing pain and deformity especially in endemic countries like India to avoid delay in diagnosis.
Conclusion: PMultifocal extensive spinal tuberculosis with tubercular arthritis with chronic kidney disease without any constitutional symptoms is rare and can mimic other benign or neoplastic etiology.
| PM0022: An uncommon case of SLE with cirrhosis and hypersplenism | |
Kishore Kunal, Singh Jasjit, Manoj Kumar, Samandeep Singh; Command Hospital Western Command, Panchkula, Haryana, India
Background: The association of systemic lupus erythematosus (SLE) with gastrointestinal autoimmune diseases is rare, but has been described in the literature, mostly as case reports. We describe here a case of SLE who presented with cirrhosis of liver with hypersplenism leading to life threatening refractory pancytopenia.
Case Report: Our patient is a 30-year-old male, who was admitted with history of high grade,intermittent fever of 15 days duration associated with episodic epistaxis and easy bruisability. Clinically he had pallor with subcentimetric cervical lymphadenopathy, multiple ecchymotic lesions on extremities and moderate splenomegaly. Lab investigations revealed pancytopenia, transaminitis, positive direct Coombs test and polyclonal hypergammaglobulinemia. His infective screen was negative. CECT of abdomen revealed splenomegaly with ascites and bilateral renal infarcts His immunological workup revealed positive ANA ( 4+ speckled, 1:640), ds DNA positivity (ELISA), low complements and Anti-SS-A, SS-B, U1RNP on ENA. His viral markers (HbsAg/Anti-HCV), ASMA/Anti-LKM/AMA was negative. His workup for APS was negative and bone marrow studies was suggestive of trilineage cellularity with megakaryoticthrobocytopenia. He also underwent transjugular liver biopsy and UGI endoscopy. His liver biopsy sample was suggestive of cirrhosis and had mild portal hypertensive gastropathy on endoscopy. He was managed as a case of SLE with secondary cirrhosis of liver and pancytopenia with IV methylprednisolone, IVIG and oral steroids. He had poor response to therapy and continued to have severe neutropenia (ANC – 700/uL). He was also given Inj GM- CSF and Eltrombopag but with no favorable outcome. He finally underwent splenectomy with gradual recovery of cytopenias.
Conclusion: Autoimmune GI manifestations is common in patients of SLE, but rarely serious and life threatening. This case report highlights the rare association of cirrhosis of liver with SLE and hypersplenism leading to life threatening refractory pancytopenia with favorable outcome after splenectomy.
| PM0023: Pseudo-obstruction in SLE: Our experience | |
Sandeep Yadav, C Balakrishnan, Rohini Samant; PD Hinduja Hospital and Research Center, Mumbai, Maharashtra, India
Introduction: Gastrointestinal involvement (GI) involvement is reported to occur in 50 % of patients with systemic lupus erythematosus (SLE) (1). Systemic reviews of literature have yielded not more than 42 cases of Intestinal Pseudo-obstruction (IPO) in SLE (2). We present our experience with 9 patients of SLE with IPO and discuss their epidemiology, clinical features, and clinical outcome.
All 9 patients were female with median age at diagnosis of 28 years. Most common presenting symptoms were abdominal pain, nausea, vomiting and diarrhea. Seven out of nine patient had chronic diarrhea while none had constipation. Median duration of delay in diagnosis was 6 months. Five of the seven patients misdiagnosed at the time of presentation and underwent avertible laparotomies. Ileum was most commonly involved (9/9) followed by duodenum (3/9), stomach (3/9) and large intestine (2/9). Supportively, colonic biopsy was negative in 7 out of 9 patients. All patients were ANA positive with ds-DNA being positive in 6/7.
CT abdomen was done in all cases showing diffuse circumferential wall thickening of the involved bowel part with dilatation with mucosal edema while 2 patients had concurrent uretero-hydronephrosis. 6 /7 patient had associated hematological and renal involvement. Available histopathology typically showed non-specific inflammation and atrophy of the muscularis layer.
All patients were treated with pulse methylprednisolone followed by oral prednisolone. Five patients had concurrent lupus nephritis, of whom three received cyclophosphamide and two received Mycophenolate. All patients had favorable response to treatment with resolution of GI symptoms maximum by six weeks.
Conclusion: Intestinal pseudo-obstruction is a rare, enigmatic manifestation of SLE. Ileal involvement is typical. Dilated thickened bowel loop is seen on imaging. Histopathology shows non-specific inflammation with atrophy of muscularis layer. High clinical suspicion and typical radiology may help in preventing a laparotomy. Response to steroids and steroid sparing agents is usually good.
 | Click here to view |
| PM0024: Atypical persistent skin eruption and a rare disease journey: Difficult to diagnosis and difficult to treat: Stills her life | |
Biswajit Banik, Debojyoti Ray; R.G.Kar Medical College, Kolkata, West Bengal, India
Adult onset still's disease is a rare peculiar rheumatological febrile illness with persistent fever; rash and arthritis.A 29 yrs female patient presented with persistent fever, persistent pruritic erythematous maculo-papular rash for 10 days; with associated symmetrical polyarthritis mainly in upper limbs joints.
 | Figure 1: Persistend pruiti c morbiliform maculo-papular skin eruption Click here to view |
 | Figure 2: Haemophaghocytes in bone marrow Click here to view |
After admission in our hospital, after battery of tests, reveled; persistent leukocytosis, high serum triglycerides, mild splenomegaly, multiple lymphadenopathy, high ESR; all routine; infective panel shows, negative result.
After considering, history and routine tests, blood for serum ferritin level and skin biopsy was done. Surprisingly, ferritin 12700 ng/ml; skin biopsy- from lesions, shows, dyskeratotic cells in the epidermis with inflammatory infiltrate in superficial dermis.
RA FACTOR, ANTI CCP, ANA (HEP 2) CELL LINE, ANCA negative.
Neoplastic screening: negative.
Bone marrow: normal study.
Treated with oral prednisolone and NSAIDs. With diagnosis of AOSD (adult onset still's disease).
After 2 months, with treatment, patients, complaining of, persistent fever, arthritis with respiratory distress. Routine investigation revealedpancytopenia, hepatosplenomegaly bilateral fluffy opacities, ferritin 22000 ng/ml; keeping in mind macrophage activity syndrome (MAS); all infected focus are screened, and bone marrow was done.
Tests revealed, hemophagocytes in bone marrow and dengue IgM was reactive; dengue PCR also shows same result. We started pulse methyl prednisolone, intravenous immunoglobulin; for 2 days; further deteriorating, the clinical profile, we started plasmapheresis and prednisolone; after 7 days, patient response to treatment, and discharged with tab cyclophosphamide, tab prednisolone, tab sulfasalazine, tab methotrexate and tab folic acid.
Discussion: AOSD is a rare systemic infection; the diagnosis made by exclusions after possible infective, neoplastic etiology rule out; without typical skin lesions it causes a huge diagnostic diplomacy.
Conclusion: Atypical skin eruption in still's disease causes diagnostic problem with treatment the course may not favorable; can transforms into MAS; dengue can precipitate the course.
| PM0025: Idiopathic necrotizing autoimmune myopathy | |
Chanaveerappa Bammigatti, Deepanjali Surendran; JIPMER, Puducherry, India
Background: We present a case of idiopathic necrotizing autoimmune myopathy, a rare entity in inflammatory myopathy.
Case Report: 32-year-old male, a ward assistant in local hospital from Kanchipuram occasional alcoholic with no comorbidities presented with c/o difficulty in getting up from the sitting and squatting position for 20 days.Later noticed swelling in bilateral forearm and calf region with upper and lower limb body pain. He felt difficulty in lifting the hand above the head and difficulty in combing hairs.no obvious muscle weakness,cranial nerve involvement, skin lesions and joint pain.no h/o drug /native medication intake.O/E power in bilateral shoulder, hip and knee joint is 4-/5 with 80% hand grip. Diminished DTR's noted. Lab investigations shown creatine kinase of 16,628 IU/L(markedly elevated),otherwise normal RFT,LFT and TFT. EMG shown positive sharp waves with fibrillatory potentials,s/o myopathic process.We proceeded with muscle biopsy(left vastus lateralis) and started on prednisolone 1mg/kg/day. Later muscle biopsy reported as necrotizing autoimmune myopathy.HPE shown early necrotic and regenerating fiber [Figure 1]. Other investigations such as ANA is negative, myoblot for anti-SRP is negative and anti-HMGCR was not done.CECT chest and abdomen is normal.Later patient is added on azathioprine 50mg BD.
 | Figure 1 Click here to view |
Discussion and Conclusion: Immune mediated necrotizing myopathy, new and a rare variant present as acute onset, symmetrical, proximal more than distal weakness.Difficult to treat than DM/PM.Correct diagnosis and aggressive therapy is required.
| PM0026: p-ANCA positivity in NMO-SD patient: Is vasculitis associated with NMO-SD | |
Sumit Kumar Vishwakarma, V P Pandey, Archana Verma; Department of Medicine, MGMMC, Indore, Madhya Pradesh, India
We are presenting 2 cases of NMO- SD with p –ANCA positivity. In about 40% cases, NMO – SD is associated with vasculitis. It gives new horizon of management in NMO -SD patients.
1st case-45-year female presented with recurrent history of paresis;
1st episode as right hemiparesis in 2008; MRI Brain -left mid brain, pons and dorsal cord hyperintensity.
2nd episode as paraparesis in 2011; MRI Brain and Spine - Bilateral Centrum semi ovale , C3-C4 and D4 -12 -T 2 HYPERINTENSITY.
VEP-prolonged
CSF-OCB, NMO-Aquaporin -4, MOG-Negative
ANA And p-ANCA-Positive
C-ANCA-negative
On the basis of criteria of NMO –SD, she was diagnosed as same.
2nd case-17-year girl presented with recurrent episode of paraparesis and seizure since 2011.
MRI Spine (2011) - Diffuse T2 hyperintense signal involving C1 to conus.
MRI Brain (2014)– Multiple new T 2 ,FLAIR hyperintensity in pons, midbrain ,right thalamus and peri and supraventricular white mater, splenium of corpus callosum and bilateral internal capsule.
MRI Brain - (2018) –New onset lesion in similar areas.
CSF-OCB -Negative
VEP- B/L Prolonged
CSF-Aquaporin 4 positive.
ANA and p-ANCA-Positive
Discussion: NMO-SD is associated with vasculitis in 40% cases, like SLE, Sjogren's syndrome and p-ANCA associated vasculitis. In our 2 patient we got p- ANCA positivity without any other clinical manifestation.
Among the CNS demyelinating disorders higher occurrence of ANCA in patient with NMO- SD than in MS, ANCA is a potential marker of autoimmunity in CNS demyelinating disorders.
We treated both the patient with Rituximab and they showed substantial clinical recovery.
Conclusion: We present 2 cases of NMO- SD with p-ANCA positivity which is not much reported in literature .These patients are in close follow up to see for clinical manifestations of p –ANCA associated clinical symptoms.
| PM0027: PUO and persistent disease activity in a child with SLE- time to think of coexisting evils | |
Prem Kumar, Sarala Premkumar, Mahesh Janarthanan; Department of Rheumatology, Division of Pediatric Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Background: SLE in children requires aggressive treatment to keep the disease under control. But the low immunity state can lead to serious infections.
Objective: We describe a 12 year old girl who presented with persistent fever and disease activity for a year. On investigation she was found to have features of military TB on chest X-ray, granulomas in the spleen and Growth of mycobacterium tuberculosis from the bone marrow and biochemical and lab features of macrophage activation syndrome.
Methods: 12-year-old developmentally normal female child, a known case of SLE with Lupus nephritis was admitted in SRMC with complaints of intermittent fever for one year and significant weight loss in August 2019. Child was diagnosed to have SLE with Lupus nephritis in 2016 at a private hospital and started on steroids but drug compliance is poor. History of Ayurveda medication intake for 2 years. On admission her vitals were stable and clinical examination showed hepatosplenomegaly and hyperpigmented macules over face and abdomen with significant muscle wasting. With a provisional diagnosis of PUO, child was treated with i.v antibiotics and supportive measures. CBC showed pancytopenia and ESR elevated. LFT was minimally deranged. Ferritin was elevated. Blood and urine cultures done showed no growth and hence i.v antibiotics stopped. Chest X-ray done showed reticulonodular opacities in both lung fields. CECT Chest done showed features of Miliary tuberculosis. Sputum smears showed AFB. Bone marrow aspirate culture showed growth of Mycobacterium tuberculosis. TB QuantiFERON test done was positive and ultrasound showed features of splenic granuloma. During the course of stay, child developed fall in hemoglobin, platelets and leucocytes with persistent fever spikes and elevated ferritin levels. Macrophage activation syndrome and disseminated TB were identified as the underlying problems and the child was treated with IVIG and methyl prednisolone and ATT. Fever spikes gradually came down and child showed clinical improvement.
Conclusion: Tuberculosis must be considered in children with SLE on significant immune suppression and persistent fevers.
| PM0028: Wegeners granulomatosis associated with unilateral facial nerve palsy- A rare case presentation | |
Abhishek Agrawal, V P Pandey, Sanjay Dubey; M.G.M. Medical College and M.Y. Hospital, Indore, Madhya Pradesh, India
Case Report: A 55 year old non diabetic non hypertensive female comes with complain of deviation of angle of mouth to the right side and difficulty in closing left eye since 5 days.
- She has history of recurrent upper respiratory tract infection with rhinorrhea (3-4episode per month) since last 2 years and history of hemoptysis 2-3 episode in last one year
- No history of fever, weight loss, contact with patient of tuberculosis, hyposthetic patch over body, skin rash, local trauma, or any other chronic illness like DM or hypertension.
On Examination-
- Pulse- 86/min, BP- 124/80 mmHg, SpO2- 98% on room air, respiratory rate- 18/min, temperature- 98.7 F
- Neurological examination revealed lower motor neuron type of left 7th nerve palsy. the rest of neurological and other systemic examination was normal
- Otoscopy examination- normal.
Laboratory Investigations-
- Haemoglobin-9.9 g/dL, TLC- 7000/Cumm, platelet-2.8 lacs/Cumm, Peripheral smear- normocytic normochromic picture
- ESR- 78mm
- S. creatinin-0.79mg/dL, s. urea-32mg/dL
- S.total bilirubin 0.20mg/dL, s. protein-6.5mg/dL, s. albumin- 4.0 m/dl, lipid profile- normal
- URIN R/M- 8-10 RBC/hpf
- HIV/HBSAg/HCV- negative
- C-ANCA- positive (>100 U/ml)
- RA FACTOR/ ANA- Negative
- C3 & C4 level- normal
- Sputum Afb- Negative
- X-RAY CHEST-Small nodules present in left lung, wedge shaped opacity in middle zone of right lung
- CT CHEST- Suggests- small nodular opacity in left lung parenchyma
- MRI BRAIN- No abnormality detected.
Patient refused for any biopsy.
Patient was managed initially with pulses of daily iv methylprednisolone and followed by iv cyclophosphamide. we also put her on azathioprine.
Discussion: Our patient presents with lower motor neuron type facial nerve palsy and after ruling out other causes of facial nerve palsy we explore the history of patient and reached the final diagnosis of wegner granulomatosis.
Conclusion: Wegener's disease can present with facial nerve palsy also so always look for features of this disease for timely diagnosis.
| PM0029: Familial hypercholesterolemia with arthritis | |
Venkatesh Yellapu, Prasanta Padhan, Sakir Ahmed; Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India
Familial Hypercholesterolemia is a disorder of lipoprotein metabolism characterized by elevated cholesterol, low density lipoprotein cholesterol, xanthomas and early onset atherosclerosis. Arthritis and tendinitis were rare in familial hypercholesterolemia. So there were often confused with reactive arthritis and other causes of polyarthritis .Here we present a case of young boy who was diagnosed as probable hypercholesterolemia with hypercholesteremic arthritis.
Report of the Case: 19 year old male came with complaint of swelling of joints of hand and feet since 8 years. He had e yellowish hard nodules all over the body since birth which were increasing in size with age. On examination there was boggy swellings in multiple joints of hand and foot and yellow nodules on elbows,knee and on buttocks. Lab investigations revealed serum cholestrol-504mg/dl,serum HDL cholesterol 25mg/dl serum LDL cholesterol 42mg/dl.On Punch biopsy of lesion were found to bexanthomas. Radiograph showed cystic and erosive lesions of metacarpophalangealjoints.
Conclusion: Metabolic disorders are often encountered in clinical practiceof these disorders are associated with musculoskeletal and dermatological manifestations. Familial hypercholesterolemia can give rise to variosmusculo skeletal disorders such as mono,oligo,polyarthritis and migratory arthiritis.Therefore it is important to recognise the association between musculoskeletal manifestations and hyperlipidemia for diagnostic and therapeutic purposes.
| PM0030: Autoimmune pancreatitis in IgG4 syndrome with Type 1 diabetes mellitus: A rare case presentation | |
Abhishek Agrawal, Abhimanyu Nigam, V P Pandey, Sanjay Dubey;
M.G.M. Medical College and M.Y. Hospital, Indore, Madhya Pradesh, India
Case Report: A middle aged nonalcoholic and non-hypertensive female comes with complain of-
- abdominal pain since 1 week
- vomiting since 4 days
- no past history of DM/HTN or any chronic illness
- No significant family history
On examination-
Pulse- 82/min, BP-126/80 mmHg, SpO2-98% on RA, RR- 18/min.
Per abdomen-Epigastric tenderness present, no guarding and rigidity per abdomen.
Mild pallor and pedal edema present.
Reddish brown rash over both cheeks, nose and forehead with sparing of nasolabial fold.
rest systemic examination was normal.
Laboratory Investigations-
- Hb- 8.3 gm/dL, TLC-3200/Cumm, platelet- 1lac/Cumm, ESR- 38/mm, RBS- 216 mg/dL, FBS-164mg/dL, PPBS- 258mg/dL HbA1C-5.6
- S. amylase- 807U/L, S. Lipase- 695 U/L
- RFT, LFT & Lipid profile- NORMAL
- T3-1.38ng/mL, T4-7.93microgram/dL, TSH-12.12 microIU/mL, AntiTPO- Positive.
- ANA- Positive
- Anti-Ribosomal antibodies- Positive
- USG abdomen- heterogenous pancreatic echotexture with mild ascites suggestive of Acute pancreatitis
- Urine R/M- proteinuria- (2+), 24-hour urinary protein- 550mg/24 hour so a renal biopsy planned. Renal biopsy suggests-lupus nephritis class II
- C-peptide- 0.4ng/mL (0.5-2ng/mL) suggests type 1 DM
- IgG4 level-3.8g/L (0.03-2.0g/L).
Discussion: Autoimmune pancreatitis (AIP) is a distinct form that can present with nonspecific symptoms like abdominal pain, vomiting and jaundice. type 1 AIP is associated with multiorgan involvement named IgG4 related disease. Association with other autoimmune diseases is common in type 1 Autoimmune Pancreatitis.
On the basis of above findings patient diagnosed as a case of-
Autoimmune Pancreatitis with type 1 DM- IgG4 disease,
SLE with Lupus nephritis and Autoimmune Hypothyroidism
Patient was treated with prednisolone, subcutaneous insulin, cyclophosphamide pulse therapy, levothyroxine supplement.
Conclusion: Whenever patient presents with acute pancreatitis and Type 1 DM, with no underlying chronic pancreatic damage, one should suspect for autoimmune pancreatitis and IgG4 correlation is to be evaluated.autoimmune pancreatitis is not related to only involvement of exocrine functions, endocrine functions of pancreas should also be looked for.
| PM0031: Ankylosing spondylitis a rare cause of peripheral nephropathy | |
Nischal Modak, V P Pandey, Monika Porwal;
MGM Medical College, Navi Mumbai, Maharashtra, India
Objective: Presenting ANKOLYSING spondylitis as a rare presence of peripheral neuropathy.
Introduction:
Ankylosing Spondylitis (AS) is an inflammatory disorder of unknown etiology that primarily affects the axial skeleton; peripheral joints and extra articular structures are also frequently involved. The disease usually begins in the second or third decade; male to female ratio is 3:1. Common sites of joint involvement are sacroiliac joints, spine, ischial tuberosities and heels. The most common extra articular manifestation is anterior uveitis (40%). Peripheral neuropathy is a rare presentation.
Objective: Ankolysing spondylitis a rare cause of Peripheral nephropathy.
Material study- 28 year old Hindu, male nondiabetic non hypertensive ,labor by occupation presented with chief complaints of low back pain since 3 years ,B/L knee and ankle pain since 2 years and losing of slippers since 6 months and difficult to hold cup of tea since 6 months followed by difficulty in walking stairs followed by difficulty in combing hairs since 6 months. On examination Pain over sacroiliac region. Normal higher mental function normal cranial nerve. Motor system - Atrophy of all of all 4 limbs, power 3 / 5 in all 4 limbs with hypotonia in all 4 limbs .Planter refl2x mute and all reflex +1.sensory system glove and stocking sensory loss. With intact bowel and bladder.Schober test positive .On Inx Normocytic normochromic anemia. NCV polyneuropathy with axonal degeneration was raised with raised ESR,HLB27 positive.ANA positive with Normal B12,Toxic screen. Therefore, diagnosis of ankylosing spondylitis was made with peripheral neuropathy.
| PM0032: A child with recurrent and refractory angioedema | |
M Sabarinath, T N Tamilselvam, N Balakrishnan, Karthikeyan, N Sujatha, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India
13/female child presented with history of recurrent episodes of swelling on face and erythematous rashes over palms, soles for the past 3 years. Child also had history of recurrent episodes of fever and multiple joint pain, 3 years past during the initial phase of her illness. There was no history of photosensitivity, oral ulcers, frothy urine and pedal edema. Child underwent evaluation multiple times in nearby hospital for facial swelling and managed with antihistamines, steroids with response to treatment
in 3-5 days. At 11 years of age child had acute abdomen pain and underwent emergency appendicectomy. On examination during present admission, child had angioedema involving periorbital region and bilateral cheeks [Figure 1]. Child also had purpura over palms and erythematous macules in pressure areas of clothing like waist line [Figure 2]. Her complete blood counts showed Hb 10.3gm/dl, TC 10500, PLT 2.5lakhs and with normal renal and liver function test. Her urine routine and PCR were within normal limits. Her ENA profile showed positive dsDNA and normal complements. Her C1 esterase inhibitor levels were normal. She was diagnosed as case of childhood lupus presenting with recurrent angioedema and cutaneous vasculitis. She was managed with antihistamines, steroids, HCQ and MMF. On follow up after four months of treatment, child had two episodes of angioedema with minimal response to antihistamines, steroids and subsiding completely with FFP transfusion [Figure 3]. We present this as a case of childhood lupus with predominant symptoms of angioedema and having poor response to immunosuppression. A literature review of SLE presenting with acquired angioedema was done. There were several case reports pointing to multifactorial etiology for angioedema in SLE with varying response to pulse steroids, antimalarial, azathioprine, danazol and plasmapheresis.
 | Figure 1: Angioedema treatment Click here to view |
 | Figure 2: Lesions in waist line Click here to view |
 | Figure 3: Angioedema after Rx with FFP Click here to view |
| PM0033: Myositis as initial presentation of sarcoidosis: A rarity | |
Mayank Gupta, Neeeraj Jain, Lalit Duggal, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India
Case Report: A 39-year-old male patient consulted in Rheumatology OPD with chief complaints of localized swelling over left calf for 6 months. Swelling was more on inner side, associated with calf fullness, without raised local temperature and muscle weakness. On investigations, Hemogram, CPK, liver and kidney function tests were normal. Myositis profile was also done which was negative. MRI of left thigh muscles showed hyperintense signals predominantly in medial head of gastrocnemius. Chest X ray showed reticular opacities in Rt. middle and upper zone. Serum ACE level was normal, Mantoux and QuantiFERON gold test were negative. CECT Chest revealed symmetrical nodules in both lungs with multiple enlarged lymph nodes in hilar,paratracheal region.Muscle biopsy was done from calf muscle which showed intrafascicular and perifascicular epithelioid granulomas without caseous necrosis i.e. in favor of sarcoidosis. MRI of left thigh and calf muscle revealed hyperintense signals in multiple muscles predominantly in medial head of gastrocnemius with thin layer of fluid around muscles suggesting Infective? Inflammatory myositis. So, based on clinical history and investigations, diagnosis of Granulomatous Myositis due to Sarcoidosis. Prednisolone 40 mg daily was started with gradual tapering of dose along with Azathioprine 50 mg twice daily. Patient responded well to treatment with reduction in size of swelling and improvement of symptoms.Muscles are involved in up to 5 % of patients with <1 % are presented as initial musculoskeletal involvement. In our case, muscle involvement was the initial and only manifestation of sarcoidosis with lung affection being picked up on routine screening.
Conclusion: We must consider rare and atypical causes of focal myositis. Tissue biopsy is must for making a diagnosis. Rare presentation of rare disease should be kept in mind.
| PM0034: A case series of malignancies: Mimics of Rheumatological disorders | |
Jeet Patel, Lalit Duggal, Neeraj Jain, Mayank Gupta, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India
Background: Malignancy and Autoimmunity interplay is full of complexities. We here, describe few cases of malignancies presented as mimics of autoimmune disorders. A case of acute myeloid leukemia had leucocytoclastic vasculitis, positive ANA, positive SS-A, and few clinical features of autoimmune disease and it mimicked for UCTD. Second case of signet ring cell type of adenocarcinoma of G.I tract mimicked CTD as had ANA positivity and ILD. Third case was epithelioid sarcoma which mimicked pyomyositis while fourth case was multiple myeloma presented with leucocytoclastic vasculitis, positive ANA, malar rash and urinary sediments and it had mimicry of SLE. Malignancy can present as an autoimmune disease or it can mimic flare of Rheumatological diseases. Cancer can present as vasculitides, CTDs, myositis or arthritides. Sometimes, it is difficult to ascertain whether autoimmune disorders have caused cancers or malignancy has caused autoimmune disturbances. ANA can be positive in upto 27% cancer patients. There is clearly increased risk of cancers amongst patients of autoimmune diseases compared to general population. Malignancies including metastasis can also present with true paraneoplastic autoimmune disorders. They usually present later in the course of the disease but sometimes surprised internist with sudden appearance in early course of disease. High index of suspicion is required to detect malignancy in autoimmune diseases as both have invariably similar presentations. PET scan and tissue diagnosis are amongst the major investigations to treat patients with spectrum of malignancy and autoimmunity
| Chiari malformation More Details, syringomyelia and polyarticular charcot arthropathy: A rare entity"> PM0035: Chiari malformation, syringomyelia and polyarticular charcot arthropathy: A rare entity | |
Jeet Patel, VedChaturvedi, Mayank Gupta, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India
Charcot arthropathies or neuropathic arthropathies are a progressive form of destructive, generally painless arthropathies. Syringomyelia has 20 to 25% risk of neuropathic arthropathy. It usually involves upper limb joints, e.g. shoulder, elbow etc. it is usually monoarticular albeit we have described here a case of polyarticular Charcot arthropathy due to syringomyelia. It can be also associated with Chiari malformations in 25% cases, particularly type one Chiari malformations. Surgical correction is advisable for Chiari malformations. No definitive treatment is available for Charcot arthropathy developed due to syringomyelia. To our knowledge, this is a first case of Chiari malformation type one with syringomyelia presented with polyarticular erosive Charcot arthropathy.
| PM0036: Subcutaneous cysticercosis of hand mimicking tenosynovitis: A rarity | |
Mayank Gupta, VedChaturvedi, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India
Subcutaneous cysticercosis in human is an uncommon parasitic infection, mostly presents as asymptomatic subcutaneous nodules with prevalence of 12.9-38% in India. It may occur as an isolated feature or as part of the disseminated cysticercosis. Here with we report a 43 years female presenting with 10 duration subcutaneous swelling. Ultrasound of left wrist showed a 5×3-mm, well-defined, thin-walled, cystic lesion with an eccentric, echogenic focus measuring around 1.5 mm in diameter in the subcutaneous plane The hypoechoic area surrounding this cyst showed significant exudative fluid collection with. The adjacent soft tissues were thickened and irregular, suggestive of edema. There was no e/o internal vascularity on color Doppler imaging. The radiological diagnosis given was a typical subcutaneous cysticercosis. Magnetic resonance imaging (MRI) evaluation of the brain which was obtained to look for disseminated cysticercosis revealed normal studies. and left-hand MRI s/o-well defined cystic lesion in muscle plane near second meta-carpal measuring approx. 5×3-mm. Extensive surrounding myofascial soft tissue swelling, and edema seen.s/o –Cysticercosis.
- Subcutaneous cysticercosis is a relatively rare form of cysticercosis but should always be considered during the evaluation of subcutaneous swellings or in suspected cases of tenosynovitis. Ultrasound is a valuable, safe, nonionizing, cost-effective, widely available imaging tool for diagnosis of subcutaneous cysticercosis.
- There is a wide spectrum of ultrasound patterns of subcutaneous cysticercosis. In classic cases with a cyst containing a scolex within and with a surrounding edema, high resolution ultrasound should always be the primary mode of diagnosis, thus avoiding unnecessary fine needle aspiration cytologies.
| PM0037: Known case of male lupus presented with acute abdominal emergency | |
Prashant Bhanjibhai Dudhagara, Alakendu Ghosh; Department of Rheumatology, IPGMER, Kolkata, West Bengal, India
Background: Thrombotic Storm(TS) is as a rare, extreme, and often lethal clinical entity characterized by a series of thrombotic events which spread over a short span of time involving the arterial and venous circulatory beds in diverse and unusual sites. There are very few case reports of Thrombotic storm successfully managed. Kidney is the most common organ involved in thrombotic storm. Involvement of liver with liver infarct and TTP makes this case rare presentation of SLE.
Case Summary: 31 year old male had history of pain in finger tips and scrotal pain with lupus panniculitis. He had history of taking steroid with HCQ and aspirin, which he stopped on his own for 2 years. Now he presented with acute abdominal pain and chest pain. All surgical cause of abdominal pain was ruled out but he was found to have liver infarcts with raised ACLA and he then developed APS nephropathy with resistant HTN and hypertensive hear failure. He also developed TTP (MAHA, thrombocytopenia, ARF,fever) for which 5 cycles of PLEX was done. He was started immunosuppressant as he had high disease activity with no signs of infection. He was put on oral anticoagulation as he had documented liver infarct. There was drastic improvement after therapy. He was fulfilling criteria of thrombotic storm. After discharge, he was given NIH protocol- 6 doses of cyclophosphamide and his anticoagulation is being continued.
Conclusion: TTP with thrombotic storm is acute emergency in a case of SLE with CAPS. Delay in diagnosisand treatment is related to high morbidity and mortality. After successful correction of thrombotic storm, prognosis of patient of SLE with CAPS.
| PM0038: Burkholderia sepsis mimicking flare of ANCA associated vasculitis: A rare presentation | |
Mayank Gupta, Lalit Duggal, Neeeraj Jain, Bhavya Chintala; Sir Gangaram Hospital, New Delhi, India
Case Report: A 54 year old lady with diabetes mellitus and hypertension presented with complaints of slurring of speech and deviation of angle of mouth towards right since 6 days and weakness of left upper and lower limbs since 3 days. She was diagnosed as AAV two years back and started on Tablet Azathioprine and oral glucocorticoids (off treatment since 8 months). Patient had cutaneous panniculitis along with high p ANCA titer not responding to above mentioned treatment; Injection Rituximab 1 gm was given. Three days later after Rituximab, patient developed above mentioned complaints. MRI spine revealed paraspinal abscess. MRI brain showed hyperintense lesions in fronto-temporal lobe suggestive of Vasculitis. CECT Chest showed multiple nodular lesions more in favor of ANCA associated vasculitis. Blood culture, pus culture of brain lesions and paraspinal abscess showed Burkhaltercepacian. Brain biopsy also revealed Burkholderia infection. She was treated with intravenous Meropenam, ventilator and other supportive treatment. Even after all possible efforts, she succumbed.
Discussion: ANCA associated vasculitis is an autoimmune condition of inflammation of small blood vessels in various organs of human body. It is a multisystem disease with protean manifestations. Burkholderia pseudomallei
s a gram negative, bipolar, aerobic, motile, rod shaped bacterium. It causes an infectious disease called melioidosis. Risk factors are Diabetes Mellitus, Chronic Renal Failure, Chronic Lung Disease, excessive alcohol consumption. Immunosuppressive patients can develop acute bloodstream infection which usually.Results: in septic shock. Neurological melioidosis is a very rare condition.CECT Chest s/o multiple, small, subpleural, randomly distributed, angiocentric, multiple cavitary and non-cavitary nodules in favour of ANCA associated vasculitis.
Conclusion: Burkholderia infection can complicate vasculitis course particularly in immunocompromised individuals. Early identification of organisms in tissue is the gold standard for diagnosis. Prompt treatment with Carbapenems and 3rd generation cephalosporins is a key to patient management.
| PM0039: Kikuchi disease with subsequent systemic lupus erythematosus: An uncommon case | |
Rashmi Bansal, Mohit Goyal1; Department of Medicine, S.M.S. Medical College, Jaipur1Department of Rheumatology, CARE Pain and Arthritis Centre, Udaipur, Rajasthan, India
Case Report: A 20-year-old female with hypothyroidism and type 1 diabetes presented with three-month history of high-grade fever and erythematosus, macular rash on upper limbs with axillary lymphadenopathy. Ultrasound revealed multiple enlarged lymph nodes with largest measuring 32x16 mm. Bloods revealed elevated ESR and CRP with negative ANA by immunofluorescence and no soluble nuclear antigens. Histopathology of biopsied lymph node revealed histiocytic necrotizing lymphadenitis suggestive of Kikuchi disease (KD). Patient improved with steroids and NSAIDs. 8 months later she presented with three-month history of hair fall, oral ulcers, joint pains, fever and erythematous rash on trunk and limbs. She had hepatosplenomegaly. ANA was positive at 1:1000 dilution with 4+ intensity and homogenous pattern on immunofluorescence. With diagnosis of systemic lupus erythematosus (SLE) she was put on tapering oral prednisolone with addition of azathioprine and hydroxychloroquine. After 5 months, patient continues to do well.
Discussion: KD is a rare, benign, immune mediated disorder characterized by cervical lymphadenopathy, fever, arthralgias and myalgias with predilection towards female gender and young age group. The disease infrequently coexists with or precedes SLE. Differential diagnoses of KD are lymphomas, lymphadenopathies associated with connective tissue disorders and bacterial or viral infections. These can be differentiated from KD on lymph node biopsy which shows paracortical foci, necrosis and histiocytic cellular infiltrate. Preservation of nodal architecture, polyclonal infiltrates and negative immunohistochemistry exclude lympho-proliferative malignancies and viral infections. The absence of neutrophils, presence of hematoxylin bodies and plasma cells with vasculitis are helpful in distinguishing SLE from KD.
Conclusion: Whilst it's important that the self-limiting KD is recognized, possibility of other diseases including SLE should be considered. Lack of markers to predict which patients progress to SLE means that patients diagnosed with KD require close follow up.
 | Figure 1 Click here to view |
| PM0040: Systemic lupus erythematosus associated with hereditary C1-inhibitor deficiency | |
Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India
Case Report: A 19-year old girl presented with the diagnosis of juvenile Systemic Lupus Erythematosus(SLE) with onset at age 11years. Her clinical features are mainly cutaneous, mild Raynaud's phenomenon, polyarthralgia and fever. Her 21-year old brother was also diagnosed with juvenile SLE at age 16years. He also had mainly cutaneous features but lately in last 6months had developed lupus nephritis.
Their 60-year old father is a known case of hereditary angioedema from age 6years. He has episodes of recurrent angioedema and acute abdomen pain. Son and daughter have no history of angioedema and father has no history of symptoms suggestive of SLE. Mother has neither of the two diseases.
Son and daughter were positive for Anti-Sm, RNP and Anti-Ro-60. Father's sera was negative for ANA and Anti-dsDNA(ELISA) was negative for all three. Serum complement C4 was low for all while son had also low C3-values. C1-esterase-inhibitor values were low for all three [Figure 1]. Next-Generation-Sequencing(NGS) showed a same frameshift mutation in the SERPING1 gene for both father and son.
Discussion: Here, we report a family with hereditary type-1 C1-inhibitor deficiency (decreased levels of C1-inhibitor) with an autosomal dominant inheritance. 23-cases of SLE associated with hereditary C1-inhibitor deficiency had been reported.[1] Defect in the inhibitor protein might lead to hyper-activation of the complement pathway, which may explain the clinical features of SLE. Both father and the son have same genetic mutation but different phenotype. This variation can be explained by some SLE-predisposing genetic polymorphisms in the early complement genes of the children inherited from the mother. Such polymorphisms is likely to be absent in father and thus protecting him from complement hyper-activity and SLE.
Conclusion: Thus, in addition to hereditary angioedema, hereditary C1-inhibitor deficiency can be associated with SLE in certain susceptible individuals.
 | Figure 1 Click here to view |
Reference
- Koide M, Shirahama S, Tokura Y, Takigawa M, Hayakawa M, Furukawa F. Lupus erythematosus associated with C1 inhibitor deficiency. J Dermatol 2002;29:503-7.
| PM0041: A case of ankylosing spondylitis with turbulent course | |
Arindam Nandy Roy, Yarram Ashok Kumar, Syeda Sana Fatima; Department of Rheumatology, Yashoda Hospital, Secunderabad, Telangana, India
Background: Hydroxychloroquine (HCQ) has shown benefits in treating rheumatic diseases such as SLE, RA and scleroderma. The risk of developing irreversible maculopathy and consequent vision loss is a possible serious complication with HCQ use. Modern day screening Methods: can detect retinopathy early in such patients.
Objective: Primary aim was to assess the prevalence of HCQ maculopathy in Indian patients with rheumatic diseases by modern day screening Methods. Secondary aim was to look for the risk factors of HCQ maculopathy.
Methods: This cross-sectional study was carried out in the Dept of Rheumatology, Yashoda Hospital, Secunderabad between July 2017 to March 2019. 984 subjects having different rheumatic diseases, who had used HCQ for 1 year and beyond and evaluated with at least Humphrey Visual Fields were included. Retinopathies other than HCQ were excluded. Informed consent was taken.
All data regarding Age, Gender, BMI, Diagnosis, Comorbidities, Daily dosage and Duration of HCQ use and Screening Methods: (Humphrey Visual Fields, Spectral Domain Ocular Coherence Tomography, Fundus Auto Fluorescence, Multifocal Electroretinogram, Fundus Fluorescein Angiography) were noted.
Chi Square test was used for statistical analysis and p<0. 05 was considered significant.
Results: Maculopathy was found in 13. 5% patients on HCQ beyond 1 year.
No statistical association was seen between HCQ maculopathy and Gender (p= 0. 189), BMI (p=0. 289), Diagnosis(p=0. 865), Comorbidities and daily dosage of HCQ (p=0. 171).
However, a significant correlation was found between HCQ maculopathy and Age {8. 4%<30 years VS 20. 3%>60 years(p=0. 033)}, Weight {8. 7%<50kg VS 15. 1%>60kg(p=0. 045)}, HCQ duration {11. 7%<5 years VS 21. 1%>5 years (p=0. 002)} and Cumulative dose {283. 79 g VS 231. 33 g (p=0. 006)}.
The detection rates of maculopathy by different Methods: were HVF (13. 5%), SDOCT (13. 3%), mfERG (12. 8%), FAF (12. 1%), FFA (11. 8%), HVF+SDOCT (13. 3%), HVF+mfERG (12. 8%) and HVF+FAF (12. 1%).
Conclusion: Hydroxychloroquine maculopathy is not infrequently seen in rheumatic disease patients in India.
| PM0042: Case of refractory diffuse alveolar hemorrhage in systemic lupus erythematosus: Role of intrapulmonary human recombinant activated factor VII therapy | |
Prakash D Paymode, V Sarath Chandra Mouli, Shilpa Suvarna, Srisaila Datta; Department of Rheumatology and Immunology, Krishna Institute of Medical Sciences Hospital, Secunderabad, Telangana, India
38 year old female, presented with cough with hemoptysis, dyspnea for 2 weeks and a fever episode 2 days before with past history ofpolyarthritis and skin rash. On examination, patient had tachypnea, tachycardia, pallor, bilateral basal crepitations with normal Blood pressure and SpO2 95%. Evaluation revealed severe anemia, ANA-IF 4+, very high Anti ds-DNA, low complements, proteinuria (1600 mg/day). HRCT chest showed bilateral diffuse ground glass opacities with interstitial opacities [Figure 1]. Her infective workup was negative. As per ACR/SLICC criteria she was diagnosed as SLE with lupus nephritis and diffuse alveolar hemorrhage (DAH). She was treated with Methylprednisolone pulse (1000 mg for 5 days), cyclophosphamide (500 mg),IV immunoglobulin(2g/kg), plamapheresis (4 cycles), Rituximab later. Despite this, DAH was progressive in the form of frank hemoptysis (200 ml/day), falling saturation and Hb. After reviewing literature, we administered intrabronchial humanrecombinant activated factor VII(rFVIIa) initially 3 mg followed by 4 mg after. Next day, hemoptysis reduced and stopped after 2nd dose. Her Hb and oxygenation improved significantly. She was continued on prednisolone (1mg/kg), cyclophosphamide fortnightly. Her DAH resolved completely. Unfortunately, on follow up after a month she succumbed to death due to sepsis.
Discussion: DAH in known complication of SLE with high mortality. In literature, in few case reports, local intrapulmonary therapy with one or more doses of recombinant Factor VIIa was found to have a good to excellent hemostatic effect by forming rFVIIa-tissue factor complexand improved oxygenationin patients with DAHwithout systemiccomplications. 1, 2. We present this case of refractory DAH responded to this newer therapy, adding to literature.
Conclusion: Intrapulmonary rFVIIa therapy is very effective should be tried in refractory DAH.
 | Figure 1 Click here to view |
| PM0043: Systemic chronic capillary leak syndrome associated with serum Anti-SSA/Ro antibodies | |
Anuj Shukla, Priyanka Gaur; Niruj Rheumatology Clinic, Ahmedabad, Gujarat, India
Case Report: 31year old lady presented with generalized edema from 20days. On examination, she had puffiness of face and pitting pedaledema. Investigation showed low serum albumin 3gm/dl, high globulin 3. 5gm/dl without proteinuria. There was no evidence of thyroid, hepatic or heart dysfunction or allergic manifestations. Her ANA was 4+nuclear speckled 1:100 with anti-Ro60(92AU/ml) and Anti-Ro52(94AU/ml) positive. Her ESR (25mm at 1hour) and serum C-reactive protein (0. 75mg/dl) were slightly raised while complements C3(0. 74gm/l) and C4(8mg/dl) were persistently low. She had no other features of Sjogren's syndrome.
The symptoms waxed-waned but persisted for 3months. Based on these features, she was diagnosed as a case of systemic chronic capillary leak syndrome (CLS). Her protein electrophoresis showed low albumin with polyclonal gammopathy but no paraproteinemia. So Anti-SSA/Ro was suspected to be the culprit. Anti-SSA/Ro persistent positivity was confirmed by ELISA 116U/ml(>15positive).
She refused regular treatment with immunomodulators and continued home remedies. She revisited after 10months and was 16weeks pregnant with complaint of increased edema from last 1month. She is now treated with hydroxychloroquine 5mg/kg and counselled regarding the risks.
Discussion: Systemic-chronic-CLS is characterized by capillary endothelial dysfunction causing extravascular leakage of fluid and small proteins example albumin resulting into hypoalbuminemia, hemoconcentration and generalized edema. In about 80% of cases, monoclonal gammopathy is detected. Acute leakage can be fatal with hypotension. Other features of pleural, pericardial effusion and ascites can be seen in severe cases. In a case series of CLS associated with autoimmune disease, 3/5 had Anti-SSA/Ro positive.[1]
In such cases, these antibodies are believed to cause capillary endothelial dysfunction mediated by autoimmunity or inflammation.
Conclusion: Thus, Systemic-chronic-CLS can be a rare manifestation of autoimmunity associated with anti-SSA/Ro antibodies positivity.
References
- Guffroy A, Dervieux B, Gravier S, Martinez C, Deibener-Kaminsky J, Hachulla E, et al. Systemic capillary leak syndrome and autoimmune diseases: A case series. Semin Arthritis Rheum 2017;46:509-12.
| PM0044: SLE presenting as chronic meningitis in a paediatric patient: A case report | |
Archana Singh, T N Tamilselvam, G Nikhila, N Sujatha, S Karthikeyan; Institute of Rheumatology, MMC, Chennai, Tamil Nadu, India
Pachymeningitis is a rare clinical entity associated with infections, malignancy or rheumatological diseases. Idiopathic hyertrophic pachymeningitis has also been reported. It is characterised by thickening of intracranial dura matter, depicted by a MRI scan. SLE can present with a wide spectrum of clinical presentations and neurological involvement.
We report a case of a pediatric patient who was treated for chronic meningitis and subsequently diagnosed as SLE.
A 15 years old female patient presented with history of headache, low grade fever of 6 months duration. There was no history of blurring of vision, weakness of any limb, altered sensorium or seizures. There was no history of rash, photosensitivity, oral ulcers or polyarthritis/polyarthralgia. She was evaluated at a local hospital for meningitis and started on ATT and acyclovir empirically. But she showed no improvementand was referred to medicine department of our hospital. On examination, patient had mild pallor. Her otherwise general physical examination was unremarkable with no evidence of a CTD. Central nervous system examination was normal with no neurological deficits. Blood investigations showed ESR-45, Hb- 9. 8g/dl, TLC- 4100 cells/μl and PC- 2. 4lacs/ μl. Her blood biochemistries were normal. CSF analysis showed no cells, normal glucose and protein. CSF bacterial and fungal cultures, CBNAAT for tuberculosis, VDRL, India ink staining, VZV IgG/IgMwere negative and adenosine deaminase (ADA) levels were normal. MRI showed bilateral tentorialenhancement and pachymeningitis. Her renal, cardiac and pulmonary evaluation was normal. Based on MRI report, rheumatologist's opinion was sought. We advised for autoimmune work up and patient was started on high dose steroids. Her CRP was >6mg/dl, ANA was 4+, C3/C4 were low and anti dsDNA was 545 IU/ml. She showed significant improvement in her symptoms and was discharged.
| PM0045: Digital gangrene as the presenting clinical feature in connective tissue disorders: A case series from Rajasthan, India | |
Neeraja Vijayan, Sonu Pandit, Maya Gopalakrishnan, Gopal Krishna Bohra, Mahender Kumar Garg; All India Institute of Medical Sciences, Jodhpur, Rajasthan, India
Introduction: Digital gangrene is a known complication of connective tissue disorders (CTD), with a varied prevalence of 30% in systemic sclerosis to 5% in rheumatoid arthritis. Here, we describe 6 patients with digital gangrene secondary to CTDs and their outcomes.
Case Discussion: Of 6 patients who presented with digital gangrene, all were females, 5 had gangrene of lower limbs, and one had both upper and lower limb involvement. The underlying CTDs were: Mixed connective tissue disorder (MCTD), SLE, Antiphospholipid syndrome (APS), Rheumatoid arthritis- CREST overlap syndrome while other two patients had undifferentiated vasculitis syndrome. All patients received on pulse methyl prednisolone followed by prednisolone which was tapered to lowest possible dose over few months. All patients except one subsequently improved with no gangrenous extension, without any requirement for surgical intervention. The patient with pulmonary-renal syndrome had fulminant course, was started on mycophenolate-mofetil and further planned for rituximab, but succumbed to illness.
Discussion: Isolated digital gangrene maybe the first presentation of various CTDs. In our experience, 5 of 6 patients presenting with digital gangrene recovered with steroid use alone, not requiring other immune suppressants. Currently all five patients are doing well on follow up.
Conclusion: Prompt initiation of immunosuppression with steroids is essential in CTD associated limb gangrene. This can arrest progression and avoid unnecessary surgical interventions or limb loss.
 | Table 1: Important clinical variables in patient diagnosed with systemic lupus erythematosus related pseud obstruction Click here to view |
| PM0046: A perplexing case of primary APLA with bilateral adrenal hemorrhage | |
Rajesh Kumar, Ratul Seal; AIIMS, Bhubaneswar, Odisha, India
Antiphospholipid syndrome (APS) or Hughes syndrome is an autoimmune thrombophilic condition that have circulating antibodies against plasma proteins that binds to phospholipids and are clinically characterized by recurrent arterial and venous thrombotic events and pregnancy morbidities. Atraumatic adrenal hemorrhage (AD) leading to adrenal insufficiency is a rare, but lifethreatening presentation of APS in whichadrenal vein thrombosis secondary to immune complex accumulation causes disruption of adrenal gland outflowultimately leading to hemorrhage infarction of adrenal gland (AD). AD is a rare presentation found in 0. 4% of APS cases and conversely APS is diagnosed in less than 0. 5% of AD. Initial diagnosis and follow up in such cases is very challenging.
A 33 years old female presented with fever for 15 days, abdominal pain, low blood pressure with no history ofabnormal bleeding or any pregnancy loss. Serum cortisol levels were low and antinuclear antibodies were absent. Abdominal CT and MRI imaging revealed bilateral adrenal hemorrhage. Meticulous workup into etiology showed increased lupus anticoagulant antibodies (IgM) and b2GP1 (IgM)on two occasions 12 weeks apart which clinched the diagnosis of 'Primary APLA' after ruling out secondary causes, in accordance of the 'Sydney Criteria' and it presented with bilateral AD hemorrhage and adrenal insufficiency. Treatment was started with steroid replacement along with warfarin and other supportive measures with close monitoring. Subsequently the patient became asymptomatic, hypotension state improved and repeated imaging in last two years of follow up, showed resolution of the adrenal hematomas.
Conclusion: A high index of suspicion is required for evaluation of adrenal insufficiency and 'APLA' should be kept in mind in cases presenting with unexplained adrenal hemorrhage. Although this is a rare presentation of APLA thenumber of recent incidences are on the rise probably due to increasing use of better radio diagnostic modalities.
| PM0047: Unilateral sacroilitis in patients with sytemic connective tissue disease | |
Vignesh Mantharam, Saranya Chinnadurai, Balaji Chillukuri, Shanmugesh Selvaraj, Shankar Ramachandran, Rajeswari Sankaralingam; Department of Rheumatology, Sri Ramachandra Institute of Higher Education and Research, SRIHER, Chennai, Tamil Nadu, India
Background: Patients with autoimmune diseases are at risk of infection and malignancy. Here we present two cases of unilateral sacroilitis (Tuberculous sacroilitis in Systemic Lupus erythematosus and metastatic adenocarcinoma in Systemic sclerosis).
Case 1: A 32 year old lady, a known SLE for 4 years with history of lupus nephritis in remission and severe pulmonary hypertension came with fever and mechanical low back pain and right buttock pain (8/10) with early morning stiffness not relieving with analgesics. MRI pelvis showed right sacroilitis with surrounding muscle edema. CT-guided bone biopsy from the sacrum showed ill-defined necrotic epitheloid granulomas over the bony fragments suggestive tuberculosis.
Case 2: A 34 year old lady, a known Systemic sclerosis with ILD for 5 years, presented with chronic cough for 2 months. On evaluation, CT chest showed left upper consolidation. Bronchoscopy was normal and BAL cytology was negative for infection and malignancy. In view of non- resolving pneumonia, patient was started on empirical tuberculosis therapy. 10 days later, she developed low back pain and left buttock pain (5/10). MRI showed left sacroilitis with STIR intensities at the attachment of adductors and obturator externus. CT guided biopsy from the left sacral bone revealed moderately differentiated adenocarcinoma. PET CT showed increased metabolic uptake from the left upper lobe and also in left 3rd rib, sacral ala and posterior element of L4, mediastinal lymph nodes and left sided pleural effusion suggestive of stage IV adeno-carcinoma of lung.
Discussion: Presence of unilateral buttock pain, unilateral sacroilitis with inflammation beyond the joint line in patients on chronic immunomodulatory therapy made usdo CT-guided biopsy which revealed the diagnosis.
Conclusion: One should not ignore a patient with severe low back pain and unilateral buttock pain. All patients with unilateral sacroilitis should be evaluated for microbiological evidence of infection or histopathological evidence of malignancy.
| PM0048: Tuberculous arthritis mimicking flare of rheumatoid arthritis | |
Vaibhavi G Velangi, Ishita S Shah, Yogesh Preet Singh; Fellow in Rheumatology
Introduction: Joint infection complicating rheumatoid arthritis (RA) although infrequent is well known. Patients with RA have 4 fold-increased risk of Tuberculosis(TB). Pulmonary TB is the most common from accounting for more than 50% of cases. Musculoskeletal TB (MSK TB) involvement occurs in only 1-3%. The most common presentation of MSK TB is chronic monoarthritis and can be mistaken for flare of RA.
Index case: A 40-year-old lady with disease onset in 2013, presented to us in May 2018 with active RA. She was not on any medications. She was started on methotrexate(MTX) 15 mg/week. As left elbow was affecting her day-to-day activities it was injected with triamcinolone acetonide 40mg.
She was subsequently lost to follow-up. In the interim, she visited doctors locally for further treatment. Sulphasalazine 2g/day and deflazacort 6mg/day were added; MTX was continued at 15mg / week. Intra-articular glucocorticoid was repeated twice over a period of 3 months. The last injection was given 3 months prior to repeat visit to our centre.
She presented to us again in January 2019 with progressive increase in left elbow pain, swelling, erythema and local raise in temperature. She did not have any constitutional symptoms. Joint ultrasound findings as per [Figure 1]. Synovial fluid AFB smear and mycobacterial culture were positive [Table 1]. Anti tubercular treatment was started. At follow-up after 2 months elbow pain and swelling had reduced considerably.
 | Figure 1: Ultrasound of the left elbow. Ultrasound of the left elbow synovial thickening (up to 10mm) with minimal joint effusion, erosion of radial head and olecradnon bursitis Click here to view |
 | Table 1 Click here to view |
Conclusion: Tuberculous arthritis can mimic a flare of RA. High degree of suspicion is required for diagnosis. Presence of disproportionate local inflammation should raise possibility of underlying infectious arthritis. Constitutional symptoms may be absent. In suspected cases synovial fluid analysis for micro-organisms should bedone at the earliest for good outcomes.
| PM0049: Acute gout mimicking flare of osteoarthritis | |
Vaibhavi G Velangi, Ishita S Shah, Yogesh Preet Singh; Fellow in Rheumatology
Background: Osteoarthritis(OA)is associated with aging. The incidence of symptomatic OA is 12-16% in adults > 60yrs of age. Crystals are not infrequently observed within the articular tissues of degenerated joints. Flare of OA can be similar to attack of mono-articular gout presenting with pain, effusion and limitation of joint mobility. Acute gout attack and flare of OA can mimic each other.
Methods: This is a retrospective case series of OA patients in whom an acute attack of gout mimicked flare of OA. Patients satisfying the 2016 ACR Clinical Criteria for OA knee were included. Duration of data collection was from 2016 to 2019. The demographic and clinical data is described in Table 1. The median age was 60 years (50-75). Out of 9 patients 7 were male. The median duration of OA was 36 months (6-72) and median duration of each attack was 21days(4-60). Knee X-rays could be retrieved in 7 patients. Kellgren and Lawrence (KL)grading for knee OA is as per [Table 1]. All patients presented with acute knee arthritis. Recurrent attacks were seen in 7 out of 9 patients. Synovial fluid analysis confirmed the presence of sodium urate crystals. All patients received colchicine prophylaxis. Urate lowering therapy was started in 3 patients and are doing well on subsequent follow-ups. In rest of the cases follow-up is awaited.
 | Figure 1: Light microscopy of synovial flyuid needle shaped monosodium urate crystals Click here to view |
Conclusion: Gout can mimic a flare of osteoarthritis. It can present as isolated knee arthritis. Knee involvement can be the presenting feature of gout. Most patients have recurrent attacks. The duration of each attack ismore than the expected duration of a typical gout attack. Co-existent gout attack needs to be considered in every patient with flare of OA knee. Synovial fluid analysis for crystals helps in differentiating acute gout from flare of OA.
| PM0050: Tubercular pyomyositis in a case of polymyositis | |
Ishita Shah, Vaibhavi Velangi, Yogesh Preet Singh, Balasubramanyam Shankar; Manipal Hospital, Bengaluru, Karnataka, India
Background: Tuberculosis (TB) is a “re-emerging disease”, with increasing incidence in 21st century, particularly in immunocompromised patients. Musculoskeletal TB accounts for 10%-25% cases of extra pulmonary tuberculosis. Tuberculous myositis is rare and may mimic malignant or other inflammatory diseases.
Case Report: A 56-year-old woman presented with severe pain, localized swelling around left thigh for 3 months. There was no history of fever, loss of appetite or weight loss. Examination showed asymmetrical, tender, red indurated area of 8 cm x 7cm on posterior aspect of thigh. Power at shoulder girdle was 4/5 and pelvic girdle 3/5. Two months ago she had visited another hospital with proximal muscle weakness. Diagnosis of polymyositis was made based on elevated CPK level, electromyogram and muscle biopsy findings. At presentation to us she was on Prednisolone 20mg per day. Investigations were as per the [Table 1]. Ultrasonography and MRI findings were as per [Figure 1] and [Figure 2]. Imaging showed disproportionate left posterior thigh involvement. Biopsy of the affected muscle group showed Acid fast bacilli on smear and culture grew mycobacterium tuberculosis. CT scan of the chest, abdomen and pelvis were normal.
 | Table 1: Demographic and clinical details Click here to view |
 | Figure 1: Ultrasonography of left thigh showed bulky muscles in posterior compartment with overlying subcutaneous oedema and minimal intramuscular free fluid Click here to view |
 | Figure 2: (Sagittal section) MRI of both thighs showed the anterior compartment muscles, posterior compartment muscles, bilateral gluteus maximus, obturator internus and externus, sartorius and gracilis muscles bulky with T1, T2 hyperintensity. Involvement of left side was more than rights side and involvement of posterior compartment was more than anterior compartment suggested polymyositis with possible super added infection in the left thigh region Click here to view |
Active myositis in the setting of active ongoing TB infection was treated with intravenous Immunoglobulins. She was started on anti-tubercular treatment (ATT). Mycophenolate mofetil was added after completing intensive phase of ATT. Improvement in muscle power and reduction in the size of induration was noted at follow up.
Conclusion: In immunocompromised state typical clinical presentation of TB is lacking; constitutional symptoms like fever, weight loss can be absent. In inflammatory myopathy, infective etiology should be considered for any atypical muscle swelling with unusual tenderness or myositis not responding to standard treatment. Biopsy of the muscle or aspiration with culture confirms the diagnosis.
| PM0051: Polyarticular septic arthritis in rheumatological diseases: A case series | |
Nupoor Acharya, Shankar Naidu, Aman Sharma, Shefali K Khanna, Sanjay Jain, Varun Dhir; Department of Internal Medicine, Division of Rheumatology, PGIMER, Chandigarh, India
Background: Polyarticular septic arthritis in adults is an uncommon finding with high morbidity and mortality. Early intervention with medical and surgical treatment is required to improve outcome. Patients with rheumatological conditions are more prone to development of septic arthritis.
Objective: To study the clinical features and outcome of polyarticular septic arthritis in patients with rheumatological illnesses.
Methods: Septic arthritis wasdiagnosed on the basisof synovial fluid showing evidence of microorganismsin theform of culture positivity and/or organism on grams stain with thesynovial fluidshowing frankpus. Polyarticular septic arthritis was defined asinvolvement of two or more joints.
Results: Here we report a series of four patients with different rheumatological conditions presenting with septic arthritis involving multiple joints. One patient had systemic sclerosis, one psoriatic arthritis, one rheumatoid arthritis and had an overlap connective tissue disease. The joints most commonly involved were the ankle and the elbow joints, (involved in 3 patients). The causative organism was Staphylococcus aureus
in two patients, pneumococcus in one, and gram-negative bacilli (not cultured) in one patient. All of the patients had received immunosuppressive therapy prior to the onset of septic arthritis. All the patients received intravenous antibiotics and surgical drainage was performed in three patients. Two patients died due to sepsis and two improved after treatment with residual joint damage.Conclusion: PASA carries a poor prognosis and mortality was high among the group despite aggressive medical and surgical interventions.
 | Figure 1 Click here to view |
| PM0052: Hide-and-See: Macrophage activation syndrome in adult lupus, a frequent but under-recognized complication. Our experience from a tertiary care hospital in Chennai | |
Ramu Ramaswamy, Rajeswari Sankaralingam, Saranya Chinnadurai, Shanmugesh Selvaraj, Balaji Chilukuri, Vignesh Mantharam, Aishwarya Ramachandran; Department of Rheumatology, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Background: Macrophage Activation Syndrome (MAS) is a severe life- threatening complication of severe Rheumatological Diseases especially SLE and sJIA. It is thought to be caused by excessive activation and proliferation of T-lymphocytes and Macrophages, leading to widespread hemophagocytosis and cytokine overproduction.
Objective: To study and describe the clinical, laboratory features, complications, precipitating factors, treatment and outcome of Macrophage Activation Syndrome in cases of Adult Lupus.
Methods: A prospective observational study was conducted in a tertiary care centre between January 2018 and June 2019. Our cases were classified as MAS based on the revised HLH 2010 criteria.
Results: Among a total 110 patients with Systemic Lupus Erythematosus (SLE) admitted as inpatients in our hospital, 7 cases (6. 3%) of MAS were identified. Mean age = 28. 57 years, M:F = 0:7, mean duration of current illness = 23. 7 days, mean duration between onset of SLE and onset of MAS ranges from 3 months to 13 years. All the patients clinically had Fever and Lymphadenopathy. 2 cases had Rash at presentation. Among the laboratory features all the patients had Hematological involvement (Anemia n=7, Leucopenia n=5, Thrombocytopenia n=4), Serum Transaminitis, Hyperferritinemia, Hypofibrinogenemia, Hypertriglyceridemia. USG Abdomen revealed mild to moderate splenomegaly in 3 cases. Hypocomplementemia (n=3), High Anti dsDNA titers (n=3). Infectious screening turned out to be positive in 4 out of 7 cases. Viral serologies revealed positive IgM antibodies to CMV in 2 cases, positive IgM/IgG antibodies to EBV infection in 1 case, and Dengue IgM/NS1Ag positivity in 1 case. All patients received Corticosteroids. Cyclophosphamide was the most commonly used immunosuppressant. Rituximab was given in 2 cases. Recorded nil mortality.
Conclusion: SLE flare and Infection were common triggers of MAS in SLE. The presentation of unexplained fever, cytopenia, or liver dysfunction, with high levels of ferritin and LDH, in patients with SLE should raise the suspicion of MAS.
| PM0053: Posterior reversible encephalopathy syndrome in rheumatoid arthritis: A rare clinical dilemma | |
Hargurdas Singh, Prateek Mangal, Dinesh Yadav, Abhinav Chowdury, Raghwendra Singh, Kamal Yadav, Tanweer Ahmad, Ashis Kumar Saha, Rezual Karim; Mata Gujri Memorial Medical College, Kishanganj, Bihar, India
Case Presentation: 35 year old female presented to us with bilateral large and small joint pain with distal interphalangeal joint sparing, morning stiffness for more than 1 hour for 1year and sudden decreased vision in both eyes from 15 days. Patient was on oral methotrexate 7. 5mg/week and DMARDS for 1 month. Vision was finger counting one metre for both eyes and ocular examination could not explain it. So MRI brain was done. CEMRI showed hyperintense areas T2 and FLAIR sequence in right cerebellum, both posterior temporal & parietooccipital region with contrast enhancement, hyperintensity on ADC map without remarkable diffusion restriction suggestive of Posterior Reversible Encephalopathy Syndrome. Ra factor 32 IU/ml, Anti-CCP 83. 21RU/ml, CRP 24mg/l, ESR 48, ANA 7. 84 AU/ml, Anti-dsDNA 0. 32 OD ratio, Urinary ACR 5. 3 ug/mg. Acc to 2010 ACR/EULAR criteria she scored 10/10. Patient was diagnosed as Rheumatoid arthritis with PRES. Patient was given pulse therapy(methylprednisolone). Then put on oral prednisolone and sulphasalazine. Her vision improved significantly in 10 days, was right eye 6/24 and left eye 6/18. Repeat MRI done after 1 month showed improvement and reduction in edema.
Case Discussion: PRES is a clinic-radiological entity of diverse etiology like hypertensive encephalopathy, eclampsia, renal failure, immunosuppresive drugs, autoimmune diseases characterised by headache, visual disturbance, seizures and radiological finding of vasogenic edema especially in areas of posterior circulation. Although exact etiology is not known, it is postulated that rapid rise in blood pressure overcomes cerebral autoregulatory mechanism causing dilatation of cerebral arterioles, opening of endothelial tight junctions and cerebral edema. Treatment consist of removing drug/causative factor. Prognosis is usually benign but may lead to permanent neurological sequale.
Conclusion: PRES is rare condition. Its clinical dilemma that PRES in my patient is due to autoimmune process or oral methotrexate as just 6-7 reported cases have long drug history of methotrexate and mainly in RA. PRES due to RA has never been reported earlier.
| PM0054: Clinical manifestations and outcome of Behcet's disease: An Indian perspective | |
Siddharth Jain, Arghya Chattopadhyay, Shankar Naidu, M Valliappan1, Vishal Sharma2, Varun Dhir, Ramandeep Singh3, Rajesh Vijayvergiya4, Manphool Singhal5, S K Sinha2, Sanjay Jain, Aman Sharma; Department of Internal Medicine, Division of Clinical Immunology and Rheumatology, Departments of1Pulmonary Medicine,2Gastroenterology,3Ophthalmology,4Cardiology, and5Radiodiagnosis, PGIMER, Chandigarh, India
Background: Behçet's disease (BD) is a variable-vessel vasculitis commonly presenting with recurrent oro-genital ulceration, skin lesions and visual disturbances. Ethnic and geographical variations exist in the clinical phenotype of BD. There is paucity of Indian data.
Objectives: To establish a clinico-laboratory profile of Indian BD patients and study their outcome.
Methods: Patients of BD (2006 ICBD criteria) presenting to Rheumatology services at PGIMER, Chandigarh were recruited prospectively from July 2017. Demographic, clinical, laboratory and radiology data and treatment outcomes were analysed.
Results: 47 patients with mean(SD) age 30. 2 (10. 8) years were recruited. 39 (83%) were males. The median duration of disease at presentation was 5. 7 years (2 weeks to 15 years). 44 (93. 6%) had oral ulcers, 38 (80%) had genital ulcers, 62. 5% had skin lesions, 72%had ocular involvement while 16 (34%) had vascular disease. Amongst non-criteria manifestations, 8 (17%) had gastrointestinal involvement while 4 (8. 5%) had neuro-behcets. Amongst vascular BD, 11 had arterial aneurysms, 8 had deep venous thrombosis (including cortical venous thrombosis) whilst 3 had both. Fever and joint pains were seen in 80% and 58% respectively. The median ESR and CRP were 32 (4-90) mm and 76 (5-198) mg/L. HLA-B51 and pathergy positivity was noted in 7/13 (54%) and 2/7 (28. 6%) patients respectively. Most patients with ocular disease were managed with azathioprine +/- cyclosporine. Meselamine or sulphasalazine was used in 3 patients with GI involvement. 9 patients with arterial aneurysms required use of cyclophosphamide while one required infliximab. Adalimumab was used in 3 patients, 2 for refractory GI disease and 1 for recurrent PG-like extensive cutaneous ulcers. Three patients (6. 4%) died, 2 because of massive haemoptysis due to pulmonary artery aneurysm, while 1 had a sudden cardiac death.
 | Figure 1: MRI brain at the time of admission, MRI brain after 1 month Click here to view |
Conclusion: Male preponderance, arterial aneurysms, ocular disease and gastrointestinal involvement was much more common in our Indian BD patient cohort compared with other countries.
| PM0055: Methotrexate misadventures: A case series from a tertiary care hospital | |
Balaji Chilukuri, Sowmya Parvathareddy, Saranya Chinnadurai, Vignesh Mantharam, Shanmugesh Selvaraj, Ramu Ramaswamy, K Punnagi, Rajeswari Sankaralingam; Departments of Rheumatology and Pharmacology, Sri Ramachandra Medical College and Research Institute, Chennai, Tamil Nadu, India
Case Series: Methotrexate (MTX) is a commonly prescribed safe immunosuppressant in Rheumatology. We report 6 consecutive cases of MTX toxicity. Five cases received MTX for Rheumatoid arthritis and one case received MTX for Granulomatosis with polyangitis. These cases have been divided into 2 groups- acute and chronic. Four cases in acute group developed toxicity due to erroneously taking MTX daily (cumulative mean dose 50 mg) during initiation of treatment. Two cases in chronic group developed toxicity on stable doses of weekly MTX (weekly mean dose 20mg). Risk factors identified for chronic toxicity were old age, renal dysfunction and concomitant use of leflunomide. Clinical manifestations were oral mucositis (n=6), gastrointestinal intolerance (n=5), skin ulcer (n=1). Cytopenias observed in chronic group were more severe than the acute group. Pneumonitis was not observed in our case series. Mean folinic acid dose administered was 230 mg and 470 mg in acute and chronic groups respectively. Oral mucositis was treated with topical squish of syrup prednisolone, antacid and promethazine. Mean hospital stay was 4. 5 days and 10. 5 days in acute and chronic groups respectively. Both patients in chronic group had persistent cytopenias and prolonged illness which responded to recombinant Human Granulocyte-Colony Stimulating Factor (G-CSF) and platelet transfusion.
Discussion: MTX widely used by Rheumatologists due to proven efficacy and affordability has the potential to cause toxicity. Both acute and chronic toxicity present with similar manifestations though chronic toxicity is more severe and needs prolonged treatment. Patient compliance, awareness about its correct administration, potential toxicity, warning symptoms of MTX toxicity, regular monitoring of renal function can prevent incidence of toxicity.
Conclusion: We observed that Chronic MTX toxicity is associated with more severe mucositis and myelosupression which needs prolonged and aggressive treatment including higher doses of folinic acid and G-CSF than acute MTX toxicity.
 | Figure 1 Click here to view |
| PM0056: A case series of Bullous Lupus: Higher incidence of NPSLE and juvenile patients | |
P Akshay, G Meghna, R Pratyusha, D Phani Kumar, R Liza; Nizam's Institute of Medical Sciences, Hyderabad, Telangana, India
Objective: To study the clinical profile of patients with bullous systemic lupus erythematosus (BSLE) in a tertiary care centre.
Methods: All SLE patients presenting with generalized vesiculobullous rash from 2006 to 2019 were identified from lupus registry. Those with localized, drug induced or infectious bullous lesions were excluded. The data was analyzed with Microsoft excel.
Results: Fifteen patients were identified. Mean age was 19. 4±6. 2 years. Of them 13(86. 6%) were females and 5(33. 3%) were juvenile. Juvenile lupus accounted for a significant proportion. Thirteen patients (86%) had bullous rash as the first manifestation of lupus while 2 had mean disease duration of 1. 5 years. Lesions were predominantly located on face, trunk and upper limbs. Lower limb involvement was less frequent with fewer lesions. Mucosal involvement was seen in one. Lesions healed with dyspigmentation in 5 and scarring in 1 patient and two had secondary infection.
Extra-cutaneous manifestations were seen in 14(93. 3%) patients with neuropsychiatric lupus being most common(56%). Of NPSLE manifestations, psychosis was most frequent (55. 5%) followed by seizures (33. 3%). Psychosis and bullous rash were concomitant in six patients, psychosis preceded in 2 and developed later in one but all within 6 months of rash.
Second most common was nephritis(33%) occurring concomitantly in 3 and after a median 2. 5 years in 2. Median SLEDAI of the cohort was 10(IQR 4. 5-14).
Skin biopsy was done in 6 patients all showing subepidermal blistering and neutrophilic dermal infiltrates. Eight patients receivedcyclophosphamide, 6 IV methylprednisolone, and 1 received rituximab.
Conclusion: Bullous lupus occurs in younger lupus patients. It is associated with neuropsychiatric lupus and lupus nephritis.
| PM0057: Case of Behcets syndrome with necrotic ulceration of lip, scrotal ulcer and leucocytosis | |
Varghese Koshy, Vandana, George Koshy, Vandana Gangadharan; Command Hospital (Central Command), Lucknow, Uttar Pradesh, India
Patient is a 23 year oldmale who initially became symptomaticwith sore throat, oral ulcers and dysphagia. He also developed an ulcer over the lower lip near the right angle of mouth which gradually became necrotic with purulent discharge.
He was initially managed with IV antibiotics for one week at a peripheral hospital and subsequently transferred to Command Hospital (WesternCommand). At this centre he also developed a scrotal ulcerative lesion.
Evaluation at the tertiary centre revealed a necrotic lip ulcer along with scrotal ulcer and Leucocytosis(more than 30, 000/mm3). Biopsy from the scrotal lesion revealed pustular vasculitis.
A clinical diagnosis of Behcets disease was made.
Patient showed an excellent response to Injection Methylprednisolone pulse 1gm OD for 3 days followed by tapering oral corticosteroids and Tab Azathioprine.
There was complete resolution of his lip and scrotalulcers.
Discussion: Leuko-cytoclastic vasculitis, fibrinoid necrosis of postcapillary venules, or perivascular neutrophilic accumulations are some of the reported patterns in the early stages of the cutaneous lesions.[1] Use of high dose corticosteroid, inspite of leucocytosis, and second line immunosuppression with Azathioprine, MMF, Methotrexate or Colchicine is also required to achieve and maintain resolution of the lesions.
Conclusion: Since Behcets Disease is a clinical diagnosis, a high index of suspicion is a prerequisite to diagnosing the condition and reporting of cases with its varied and myriad manifestations.
References
- Chun SI, Su WP, Lee S, Rogers RS 3rd. Erythema nodosum-like lesions in Behçet's syndrome: A histopathologic study of 30 cases. J Cutan Pathol 1989;16:259-65.
 | Figure 1 Click here to view |
| PM0058: Gastrointestinal sarcoid: A rare initial manifestation of sarcoidosis | |
Pooja J Belani, Vishad Viswanath; Institute for Rheumatology and Immunology Sciences, Thiruvananthapuram, Kerala, India
A 30-year-old gentleman presented with 3 years history of intermittent watery loose stools, 2-3 episodes/month, 4 times a day, that was difficult to flush with inflammatory low backache, EMS of 30-60 mins and difficulty in turning in bed since 4 months. There was no weight loss, fever, recurrent oral ulcers, red painful eyes, chronic cough or breathlessness. He had past history of scalp psoriasis 6 years back improved with coal tar treatment. General and systemic examination, including MSK was unremarkable. Possibility of Spondyloarthritis was strongly considered. Hemogram and renal parameters were normal except elevated AST/ALT and S. ALP, for which USG abdomen and MRI abdomen was done that was suggestive of preaortic and paraaortic lymphnodes, with hepatosplenomegaly. Liver, gastric antrum and lymphnode biopsy were suggestive of well-formed granuloma with negative cultures for TB, negative IHC markers for lymphoma. Differentials thought were Crohns disease and sarcoidosis. S. ACE levels and calcium were normal. Provisional diagnosis of sarcoidosis was made on the basis of hepatic granuloma which is rare in Crohn's disease. He was started on tapering Prednisolone 40mg/day.
 | Figure 1 Click here to view |
Discussion: Symptomatic gastrointestinal(GI) tract involvement(excluding hepatobiliary system) is rare manifestation of sarcoidosis with prevalence of 0. 1-0. 9%. Only 44 cases of asymptomatic gastric involvement are recorded till 2010. Stomach is the most common site and small intestine is least common. Absolute pointers favouring sarcoidosis over Crohn's disease include hypercalcemia, lung involvement, elevated ACE levels, cardiac involvement and generalized lymphadenopathy. Palpable splenomegaly and hepatic granulomas are rare in Crohn's as compared to sarcoidosis. Lymphoma can rarely present as granulomatous disease. Infections like Tuberculosis, Histoplasmosis, Syphillis, and chronic EBV need to be ruled out. Steroids should be started in symptomatic patients with the addition of steroid-sparing agent in case of nonresponse or steroid dependence.
Conclusion: Sarcoidosis is great mimicker and should be considered in the differential diagnosis of any systemic granulomatous disease.
| PM0059: Myasthenia Gravis masquerading as myositis in autoimmune diseases | |
Kasturi Hazarika Manesh Manoj, Prashant Bafna, Rasmi Ranjan Sahoo, Anupam Wakhlu; Department of Clinical Immunology and Rheumatology, King George's Medical University, Lucknow, Uttar Pradesh, India
Herein, we discuss two cases of Myasthenia gravis(MG), one associated with primary Sjogren's syndrome (SS) and the other with Takayasu arteritis (TA).
Case 1: A 60yr-old female admitted with history of difficulty in walking, polyarthralgia and dry mouth for the last 2 years. She had mild proximal and distal weakness. Initial investigations revealed severe hypokalemia, with normal anion gap metabolic acidosis & alkaline urine with nephritic range proteinuria and no active sediments. Renal tubular acidosis was suspected. A positive ANA & ENA along with clinical features confirmed a diagnosis of SS. Later, she developed quadriparesis including distal muscle & bulbar weakness, despite improving hypokalemia. Myasthenia gravis was suspected and confirmed with a positive neostigmine test and positive myasthenia autoantibody profile.
 | Table 1: Laboratory tests Click here to view |
Case 2: A 35yr-old female admitted with h/o easy fatiguability since 2 years, absence of pulses of right upper limb since 8 months and generalized weakness for 3 months & symptoms suggestive of palatopharyngeal weakness for 15days. On admission, she had severe neck and proximal muscle weakness and drooping of eyelids which on further questioning had a diurnal variation. A positive neostigmine challenge and repetitive nerve stimulation test confirmed MG. The right upper limb blood pressure was elevated. A CT aortogram revealed diffuse narrowing of the infrarenal aorta including bilateral external iliac arteries and narrowing of the left subclavian artery and proximal part of left axillary artery s/o TA.
Both patients were initially managed with IVIG, low-dose steroids gradually increased to 1mg/kg prednisolone to avoid acute worsening of MG and pyridostigmine. For case 1, potassium supplementation along with correction of acidosis was done and second-line immunosuppression with mycophenolate mofetil initiated. For case 2, anti-hypertensives were added as required and started on azathioprine as steroid-sparing agent.
Conclusion: MG can complicate the presentation of a number of autoimmune conditions.
| PM0060: Myelitis in adults and children with lupus: Experience from a single tertiary care center over 25 years | |
Pankti Mehta, Latika Gupta, Hafis Muhammed, Durga P Misra, Able Lawrence, Vikas Agarwal, Amita Aggarwal, Ramnath Misra; Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India
Introduction: Myelitis can rarely occur in the setting of lupus. Understanding the prevalence, demographic profile, clinical and serologic profile and treatment outcomes can be helpful for early identification and better management.
Methods: Medical records over 30 years from 1989-2018 from a large tertiary care center in Northern India were reviewed to identify patients with various forms of myelitis. Their demographics, clinical profile, course of illness, autoantibody profile and outcomes were compared with patients of lupus without myelitis. For each case, 2 matching comparators were drawn by the hospital registration number. Categorical variables were compared using chi-square and continuous variables using students-t test. p<0. 05 was taken as statistically significant. All statistics were done using SPSS (v23, IBM 2010).
Results: Of the 10 (0. 56%) of 1768 lupus cases who had myelitis, 3 (30%) were classified as Neuromyelitis Optica- NMO, 5 (50%) had NMO-Spectrum Disorder- NMOSD, 1 (10%) as Transverse Myelitisand 1 (10%) had a Clinically isolated syndrome -CIS. In 7 (70%), Myelitis was the first manifestation of Lupus. 6 of the 10 had relapsing disease (17 events) with a median time to relapse 2. 05 years (range 0. 08–15 years). ANA was negative to start with in 2 cases. 1 (25%) of the 4 tested positive for Anti-Aquaporin 4 antibody. Nephritis (2 vs. 15, p-0. 007) and hematologic (0 vs. 8, p=0. 029) manifestations were seen less often in Lupus with myelitis than those without it. Of the 7 who followed up, all received maintenance immunosuppression with Cyclophosphamide, Azathioprine or Rituximab.
Conclusion: Myelitis can be the first manifestation of lupus. CIS although not a part of ACR 1999 criteria for NPSLE is often seen in these patients. Nephritis and hematologic manifestations are less common in Lupus with myelitis. The disease should be aggressively treated with maintenance immunosuppression to prevent relapses.
 | Figure 1: Timeline of events of all 10 patients. Steroids give for every relapse (Not mentioned separately). Steroid sparing agent mentioned in bold whenever give. Outcome mentioned on a liker scale at the extreme right corner in red. J- January, F-February, A-April, Ju-July, O-October, S-September, N-November, A-Arthritis, LETM- longitudinally extensive transverse myelitis, ON- optic neuritis, CYC-Cyclophosphamide, RTXRituximab, AZA-Azathioprine, ACS-Acute confusional state, LN-Lupus nephritis, NIH-National institute of health protocol, ELNT-Euro-lupus nephritis trial protocol Click here to view |
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