Rheumatology

PM0061: Case report of focal myositis in a patient of systemic sclerosis

G S Nikhila, T N Tamilselvam, Archana Singh, Karthikeyan, Sujatha; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

25 yr old female was admitted with pain and swelling of left thigh for 3 weeks. She was a known case of Limited cutaneous systemic sclerosis with early ILD and was on MMF 1. 5 gm/day. On examination, there was a tender swelling of 4x3 cm in anterior aspect of middle 1/3^rd of left thigh. Muscle power was normal and there was no systemic symptoms. Investigations showed elevated ESR and muscle enzymes with other biochemistry and CBC being normal. MRI left thigh was suggestive of myositis involving left Rectus femoris muscle. EMG showed myopathic pattern in left Rectus femoris, NCS was normal.

Patient was treated with oral steroids at 0. 5mg/kg/day and MMF continued. She responded to treatment with resolution of pain, swelling and normalizing muscle enzymes.

Discussion: Focal myositis is a rare benign inflammatory pseudotumour of skeletal muscle, that usually present in the extremities. Systemic symptoms and muscle weakness are rare. Diagnosis is by presence of elevated muscle enzymes, MRI imaging and muscle biopsy. Being an inflammatory condition, this responds well with steroids and immunosuppression. Focal myositis is describedin CTDs like Systemic sclerosis as one type of myositis.

Conclusion: Focal myositis should be considered when evaluating a patient with soft tissue mass in muscle plane in CTDs and is a DD for muscle sarcomas.

Figure 1 Click here to view

PM0062: Early onset rheumatoid vasculitis

Abhishek Pandey, Deepak Gautam, Lalit Prashant Meena, Ankit Mishra; Department of Medicine, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India

Rheumatoid vasculitis annual incidence among patients with rheumatoid arthritis (RA) is less than four per million. The condition usually occurs in patients with longstanding, severe rheumatoid arthritis. We are going to present a case series of ten patients presented with early onset rheumatoid vasculitis. We compared clinical characteristic, laboratory data and course in this case series.

Figure 1 Click here to view

PM0063: Atypical manifestation as an initial presenter of systemic lupus erythematosus

Meghanad Meher, Rajesh Kumar, Ratul Seal; AIIMS, Bhubaneswar, Odisha, India

Here we described 4 atypical clinical manifestation of systemic lupus erythematosus(SLE) whose prevalenceis in the range of 0. 5-2%, but as an initial presenter of SLE is very rare though there are sparsh literature.

SLE with Digital Gangrene: A 20 year old female presented to us as 2 month history of gangrene over right great toe which was preceded by pain in the toe, pain in knee, fatigue & loss of hair. physical examination revealed gangrene of right great toe. On further investigation patient was diagnosed as a case of SLEwith digital gangrene. Patient was treated with pulse methylprednisolone, oral steroid, pulse cyclophosphamide, mycophenolate mofetil, &hydroxychloroquine.

SLE with Spontaneous Hydropneumothorax: 20-year old male presented to emergency department with a history of fever for 2 months, shortness of breath & right sided chest pain for 2 days. On systemic examination and routine investigation patient was diagnosed as a case of right sided hydropneumothorax was established. On further evaluation of fever patient was diagnosed as a case of SLE. Patient was treated with intercostal chest tube drainage, steroids & hydroxychloroquine.

SLE with Macrophage Activation Syndrome: 15-year old female presented with fever, loss of hair, multiple joint pain, rash, bleeding from nose & gums. On general examination facial puffinace, malar rash, palatal ulcer& bleeding from gums was there. Onfurther investigation we diagnosed as a case of SLE with macrophage activation syndrome. patient was treated with pulse methylprednisolone, oral steroid, hydroxychloroquine & cyclosporine.

SLE with Thrombotic Thrombocytopenic Purpura: 62 year male presented to us with altered sensorium, persistent hiccups for 10 days. Examination finding reveled purpura and ecchymosis all over the body, fever, tender knees. On further investigation patient was diagnosed as a case of SLE-TTP. Patient was treated with pulse intravenous methylprednisolone, oral steroid & hydroxychloroquine.

PM0064: Anti-SRP myopathy: Case series from a single tertiary center with review of literature

Rajiv Ranjan Kumar, Saket Jha, Aadhaar Dhooria, GSRSNK Naidu, Susheel Kumar, Shefali Khanna Sharma, Aman Sharma, Sanjay Jain, Varun Dhir; PGIMER, Chandigarh, India

Background: Signal recognition peptide (SRP) is a cytoplasmic RNA protein that translocates newly synthesized protein across the endoplasmic reticulum (ER). Myositis associated with SRP is severe and refractory, may affect all ages and is usually associated with polymyositis.

Objective: We describe the clinical features, treatment, and outcomes of anti-SRP myopathy patients from a single tertiary care center.

Methods: A retrospective, single-center, study. Patients with myositis with SRP antibody positive by immunoblot and fulfilling ENMCC criteria-2017.

Results: 6 patients (all female) were recruited; mean age 49±14. 5 years. Five patients had symptoms duration of £ 12 months. All patients presented with proximal muscle weakness; two had severe muscle weakness, two each had neck weakness, dysphagia, bulbar weakness, and muscle atrophy; and one had mild distal muscle weakness. Four patients fitted in the dermatomyositis spectrum while two were consistent with polymyositis. One patient had myocarditis, none had interstitial lung disease. Muscle biopsy was done in 3 patients; one showed necrotizing inflammation while the rest showed nonspecific myositis. The mean CPK level was 1034 ± 675 IU/L. All patients received corticosteroids with added immunosuppression (methotrexate in 5, azathioprine in 1). One patient was refractory to methotrexate, steroids, rituximab, intravenous immunoglobulins, plasma exchange, and tacrolimus and expired. On follow-up (11. 8± 7 months), five patients were doing well with 2 relapses. Ovarian cancer was seen in one patient.

Conclusion: Anti- SRP myopathy is a severe form of inflammatory myositis. Our patients had a good response (barring one) with corticosteroids and conventional immunosuppression.

PM0065: Tuberculosis a mimicker, masquerader and response modifier to autoimmune diseases

Kattel Vivek, Agrawal Yamuna¹, Tilwee Virend, Gupta Neetu², Malviya Sourabh; Medanta Superspecialty Hospital,²MGM College, Indore, Madhya Pradesh, India,¹BP Koirala Institute of Health Sciences, Dharan, Nepal

Introduction: Globally almost a quarter of population has latent tuberculosis and 6-8% has autoimmune diseases. Overlap between these two conditions may present with various clinical pathology.

Cases: A 42-year home maker with polyarthritis, raised ESR/CRP, HLAB27 positive and grade I bilateral sacroilitis (SI) on X-ray pelvis on high dose steroid and methotrexate was planned for TNF blockers against recurrent then refractory left sided uveitis. One month rifampicin and isoniazid for her Monteux and IGRA positive latent TB relived her uveitis and she was prospectively diagnosed as tubercular uveitis and competed ATT course. Active tuberculosis can mimic HLAB27 syndrome (uveitis, peripheral arthritis) due to high prevalence of latent tuberculosis and asymptomatic HLAB27 positive in community.

A 36-year businessman diagnosed case of HLAB27 positive Spondyloarthritis was previously treated for latent tuberculosis on adalimumab under remission presented with right knee arthritis with ESR 141mm and CRP109U/mL.Synovial tapping revealed inflammatory arthritis and patient was changed to eternacept with addition NSAIDS and methotrexate. He worsen with calf myositis not responding to broad spectrum antibiotic. A trial of ATT drug was initiated and he started to respond by the end of a week. He continued intensive phase for two months followed by eight months continuous phase of ATT regime. Post ATT his spondyloarthritis is under remission with sulfasalazine. In our case, latent tuberculosis treatment did not prevent the reactivation of tuberculosis.

Discussion: Clinical judgment on manifestation that mimics tubercular or autoimmune diseases is tough. In high latent tuberculosis geography trial of anti-tubercular for at least month followed by assessment of autoimmune disease could answers such dilemma unless we have a test to differentiate between latent and active tuberculosis.

Conclusion: A clear affordable guideline regarding investigation and treatment of latent tuberculosis does not exist for rheumatologic patients at developing world.

Keywords: Autoimmune diseases, biological DMARDS, tuberculosis

PM0066: Immunological non responder's real or virtual phenomenon

Kattel Vivek, Agrawal Yamuna¹; Medanta Superspecialty Hospital, Indore, Madhya Pradesh, India,¹BP Koirala Institute of Health Sciences, Dharan, Nepal

Introduction: A subset of HIV people on antiretroviral therapy (ART) achieves virological suppression but poor recovery of CD4 cell termed as immunological non-responders.

Case: A 24 years intravenous drug abuser male with HCV for last three years presented as HIV positive (CD4 - 186/ml) on July 2008. Despite ZDV/3TC/EFV for six months he did not achieve immunological recovery but his viral load was below 400copies/ml. On September 2009 he presented with fever and constitutional symptoms for two weeks. On examination he was pale, icteric and had hepatosplenomegaly. Investigation revealed pancytopenia, transaminitis, hepatosplenomegaly, sterile blood culture, normal chest X ray, sputum for acid fast bacilli and PCR for mycobacterium tuberculi negative, negative rK-39, malaria negative. He had CD4 of 156/ml, HIV viral load 72copies/ml, HCV RNA 15600copies/ml. Bone marrow aspiration revealed 3+ Leishmania Donovani (LD) bodies. ARV regimen was changed to TDF/3TC/EFV and tablet Miltefosine 50 mg twice a day for 28 days was initiated. He improved clinically and parasitologically. On April 2010 his second infection of Visceral Leishmaniasis (VL) was treated with Injection Amphoteracin B. On March 2011 and August-2012 he had third and fourth episode of VL infection and was treated with Amphoteracin B plus Miltefosine and liposomal Amphoteracin B respectively. However, the fourth episode was continued with secondary prophylaxis for six months with immunological recovery (CD4 756/ml). On April 2015 his HCV was treated with 12 weeks Sofosbuvir and Daclatasvir with Rapid Viral and Sustained Viral Response.

Discussion: It has been recommended to start HCV treatment in HIV coinfection if CD4 cells are more than 200/ml. Immunological non-responders could be a challenge to initiate HCV treatment especially in limited resources setting.

Conclusion: Immunological non responders might be virtual phenomena.

PM0067: Isolated thrombocytopenia: A rare presentation of primary Sjogren syndrome

Sumit Puloria, V P Pandey, Sanjay Dubey; M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

Case Report: A 30 year female came with complaints of Bleeding per vagina since 3 month & with nonpruritic purpuric rashes over both lower limb more than upper limb since 10 days. No history of fever, joint pain or any drug intake. Vitals- stable & Systemic examination was normal.

Haemoglobin -11. 2 gm%

TLC -5100/Cumm

Platelets - 9000/Cumm

P. S. - RBC microcytic hypochromic with mild anisocytosis seen, few tear drop &pencil cells. Platelets count - inadequate.

ESR24/mm

Bone Marrow aspiration- WNL

Immature platelet fraction- 52% (increased)

Ecg;Urine R/M;Usgabdomen ; Chest x ray - All are WNL

DENGUE /HBsAg/ AntiHCV/ HIV/ Widal -Negative

R. A. factor / Anti CCP/ CRP/ ANTI LKM 1/ ANTI ASMA – Negative

Thyroid profile - WNL

ANA by IFA -SPECKLED PATTERN: 2+

ANA profile by Immunoblot -Specific band for SSA/Ro60KD, SSA/Ro52KD, SSB antibodies

SCHIRMER'S TEST: POSITIVE

• Rt. & Lt. Eye-3mm &0. 5 mm respectively
• MINOR SALIVARY GLAND BIOPSY: evidence of focal lymphocytic sailadenitis. FOCUS SCORE – 0. 73/4mm.

Pt. was managed with pulse therapy ofI. V. Methylprednisolone for 5 days & then with oral steroids, clinically her bleeding tendencies were stopped & plateletscount recovered.

Disscussion: Primary Sjogren's syndrome is an autoimmune disease wherethere is lymphocytic infiltration of salivary and lacrimal glands. The most common clinical feature of Sjogren's is dryness of the mouth and eyes. Rarely patients can present with autoimmune cytopenias. Here we report a case of a young female presenting withisolated thrombocytopenia. Incidence is 5-15%.

Conclusion: We should be vigilant enough to look for Sjogren's syndrome in patient presenting with isolated thrombocytopenia as they can have dreaded complication if not managed on time.

Figure 1 Click here to view

PM0068: HLH in SLE: A rare presentation

Sumit Puloria, V P Pandey, Sanjay Dubey, Yogendra Jamra, Karuna Mujalda; M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

A 20-yr-old female presented with fever, arthralgia, and 1 -episode GTCS.

• On examination, pulse- 80/min, respiratory rate was 16/min andBP- 110/70 mm of Hg, Temp. - 100. 1 F. General Examination reveals oral ulcers, pallor & icterus
• Systemic examination reveals generalized maculopapular rashes, sparing nasolabial fold, oral ulcers, splenomegaly
• Haemoglobin- 5. 7 gm%, TLC- 2000/Cumm, Platelets- 51, 000/Cumm, MCV- 85/fL, ESR- 45 mm/hr, Serum creatinine- 0. 99mg/dl, Total bilirubin- 8. 9 mg/dl, Direct Bilirubin- 6. 8 mg/dl, SGOT- 877 U/L, SGPT- 104U/L, ALP-269IU/L, Triglycerides- 528mg/dL, INR- 1. 24,
• HIV/ANTIHCV/HBsAg- Negative
• ANA by IFA revealed +4, Homogenous pattern
• ANA profile by Immunoblot- ANTI ds DNA - positive
• Serum Ferritin > 1650 ng/ml
• ANTILKM-1 /ANTI SMA – Negative
• Serum ceruloplasmin- 27. 2 mg/dL
• Bone marrow aspiration- Haemophagocyte were seen.

Pt. was managed with pulse therapy ofI. V. Methylprednisolone, antiepileptics, but pt. succumb within 14 hrs.

Discussion: HLH is seen rarely in patients of SLE

• Whenwe suspect to have acute flare up of SLE, a possibility of secondary HLH should be kept in mind
• In our patient, she had acute flare-up of SLE, triglycerides level were raised & serum ferritin level was sent, that also came to be highly raised, her BMA was planned that revealed HAEMOPHAGOCYTES.

Conclusion: ØSecondary HLH with SLE is rare, with an estimated prevalence of 0. 9–4. 6% and when it occurs, differentiating it from lupus flare requires a high degree of suspicion and awareness of this association.

ØEarly diagnosis of HLH in the Background of SLE facilitates the timely selection of an appropriate treatment modality to prevent fatal complications.

Reference

1. Fukaya S, Yasuda S, Hashimoto T, Oku K, Kataoka H, Horita T, et al. Clinical features of haemophagocytic syndrome in patients with systemic autoimmune diseases: Analysis of 30 cases. Rheumatology (Oxford) 2008;47:1686-91.

Figure 1 Click here to view

PM0069: Unmask GBS and discover “the vasculitis”

Adamya Gupta, Archana Verma, V P Pandey; MGM Medical College and MY Hospital, Indore, Madhya Pradesh, India

Introduction: Vasculitic neuropathy classically presents as mononeuritis multiplex, in certain patients neuropathy can be symmetric & may predate diagnosis of collagen vascular disease. such GBS if has undiagnosed association with vasculitis generally lead to poor prognosis. So if we diagnose vasculitis, treat vasculitis we treat GBS.

Materials and Methods: Case series of 23 patients came to myh in last 6 months, cases were diagnosed on basis of history, examination, routine blood biochemistry, nerve conduction studies, csf examination, & if suggestive ana was done.

Observations: we found GBS patients 82% males, 17% females young<= 35 years 73%, >35years 26% demyelinating variant 73%, axonal variant 26%

vasculitis was observed in 47% overall of which 26% were demeylinating cases & 21% were axonal variants.

66% of elderly patients (>=35 years) had vasculitis, while only 41% young patients (<35 years) had vasculitis.

40% patients with axonal variety along with vasculitis were anti- ro ab positive.

Arthralgia as a presenting complaint as well as association was seen in 8% patients overall. but every case of arthralgia with GBS had 100% specificity to be ana positive in our study.

Conclusion:

Although allof these patients appeared clinically to have GBSi. e. rapidly evolving symmetrical motor neuropathy, there is no specific laboratory test to definatively diagnose GBS. Thus there may be errors in diagnosis.

Hence several disease may mimic GBS

Thusvasculitic neuropathy should be thought as treatable cause of

• otherwise poor prognostic GBS
• worsening GBS
• GBS who have electrophysiologic or lab findings atypical of GBS like



• axonal variant GBS
• aidpa/w arthralgia
• severe weakness (<3/5 power grade).

Agressive workup like s. ana, msucle biopsy, nerve biopsy should be done to find/ rule out cause of treatable neuropathy.

PM0070: Tubercular osteomyelitis of bilateral wrist joint

Sumit Puloria, V P Pandey, Sanjay Dubey, Ashok Thakur;

M. G. M. Medical College and M. Y. H. Hospital, Indore, Madhya Pradesh, India

A 15 year old boy came with presenting complaints of swelling and pus discharge in the left wrist: 3 year & swelling in the right wrist: 1 month

On General Examination vitals – stable, pallor was present, multiple lymph nodes enlargement seen in axilla and inguinal region.

On Local Examination: multiple firm tender skin-colored to hyperpigmented swelling over wrist, elbow & axillae along with scarring and draining sinuses.

Systemic Examination- WNL

• Haemoglobin- 7. 6 gm%, TLC- 11, 700/Cumm, Platelets- 3. 5 lac/Cumm, MCV- 65/fL, ESR- 55 mm/hr, serum Creatinine- 0. 58mg/dl, Total bilirubin- 8. 9 mg/dl, Direct Bilirubin- 6. 8 mg/dl, ALP-203IU/L, INR- 1. 24, Serum proteins- 7. 1 mg/dL, albumin- 3. 0mg/dL
• HIV/ANTIHCV/HBsAg/VDRL/ RA factor - Negative
• CRP – Positive (96microgm/mL)
• Culture & Sensitivity (Blood/Urine/Pus)- sterile
• USG Neck & Axilla – few subcentrimetric lymph nodes seen in anterior triangle of neck, multiple enlarged Lymphnodes are seen in bilateral axillae largest measuring 2. 4. *1. 3 cm
• USG of Left Wrist: Approximately 2. 8 x 1. 9 x 1. 8 cm heterogenous hypoechoic soft tissue mass seen on dorsal aspect of Lt. wrist joint with destruction of underlying bone seen & adjacent bone cortex appear irregular
• MRIB/L WRIST: diffuse marrow edema noted in most of carpal bones. capitate & 4th metacarpal severely affected
• PUS CBNAAT: MTB DETECTED; Rifampicin Sensitive
• Pt. was managed with A. T. T. & analgesic, and is in follow up.

Discussion: Pt. came with discharging sinuses and multiple enlarged lymph nodes, early recognition of this disease and treatment can prevent joint deformity & functional disability.

Conclusion: Very rare form, constitutes about 1. 7–2% of all TB cases. A general physician was less aware of its presentation. . . Prompt diagnosis and treatment are of utmost importance to avoid the development of skeletal deformities and functional disabilities.

PM0071: Unusual case report on muscular dystrophy

Lakshman Meena, Danveer Bhadu; AIIMS, New Delhi, India

A 37 year female known case of hypothyroidism on thyroxine 50 microgram tablet daily from last 6 year and hypertension on tabletamlodipine 5 mg daily from last two year came to our OPD with chief complain of proximal muscle weakness of upper and lower limb last oneand half year. Power in both upper limb and lower limb was 4/5. She also give history of unable to sit from lying down position (truncal muscle weakness). There is no history of distal muscle weakness, no significant family history, no history suggestive of CTD. On evaluation TSH level was 6mU/L. CPK and LDH level was 853 U/L & 1635 U/L, EMG was normal. During evaluation she was found rounded hypoechoic exophytic lesion (4 x 4 cm) on lower pole of right kidney. PET scan show metabolically active soft tissue mass at inferior pole of right kidney, hypermetabolism of bilateral leg muscles, paraspinal muscles and right side abdominal wall muscles with diffuse atrophy –suggestive of myositis. Histopathology of kidney mass suggestive of renal cell carcinoma for which nephrectomy was done by urology department. However, EMG was normal but due to raised CPK, LDH and PET scan suggestive of myositis &renal cell carcinoma we made a diagnosis of Paraneoplastic myositis and we started Steroid 1mg/kg in tapering dose along with Methotrexate but after 2 month there is no significant improvement. So, we plan and give Rituximab 1gm at 0, 2 weeks. Even after two and half month patient have no significant improvement in muscle weakness and CPK was 721 U/L. So we do muscle biopsy which was suggestive of muscular dystrophy probable calpainopathy.

Conclusion: We should do muscle biopsy when 1. EMG and muscle enzyme finding mismatch 2. Muscle enzyme level no significantly high 3. No response with steroid and DMARDS including biologics.

PM0072: A case report of autoimmune cytopenias: A rare presentation of primary Sjogren's syndrome

V N Nagaprabu, P Velammal¹, Gayathir Anand; Sakthi Rheumatology Centre Pvt., Ltd.,¹PSGIMS and R, Coimbatore, Tamil Nadu, India

Primary Sjogren's syndrome is an autoimmune disease characterized by lymphocytic infiltration of salivary and lacrimal glands. This inflammation is thought to be caused by B-lymphocytes. The most common clinical feature of Sjogren's is dryness of the mouth and eyes, but rare complications can occur such as autoimmune cytopenia. Here we report a case of immune-mediated cytopenia's that were diagnosed to be due to Sjogren's syndrome.

Case Report: A 30-year female admitted with complaints of bleeding gums, petechial and purpuric rashes over limbs of 10 days duration.

Past history-Known hypothyroidism on thyroxine replacement therapy. History of platelet-rich plasma transfusion 2 years ago.

On examination BP-110/70 mm hg, PR-86/min, Pallor- was present

Icterus, Lymphadenopathy, and Hepatosplenomegaly were absent. ANA by IIF revealed +2. ANA profile showedAnti RO/SS-A positivity. Anti-ds DNA and anti-Smith antibodies were Negative.

The patient was treated with steroids, platelet transfusions, and IVIG.

Investigations-

Haemoglobin-6. 4 g/dl

TLC-8200 cells/cubic mm

Platelet count-4000/Cu mm

Peripheral smear-Normocytic, normochromic RBCs and normal platelets

Reticulocyte count-0. 5%

Coombs test-Negative

HIV, HBsAg and Anti HCV-Non reactive

RFT, LFT-Normal

Immature platelet fraction-55. 2%

ANA-Positive

Anti RO/SS A-Positive

Anti-LA/SS B, Anti ds DNA, Anti Smith antibody-Negative

Schirmer's test-Positive.

Discussion: Leukopenia is seen in 14-42 %, thrombocytopenia in 5-15%, and anemia in 11% of patients with Primary Sjogren's syndrome (pSS)and can manifest individually or in combination.

Treatment generally includes immunosuppressive therapy, corticosteroids and /or IVIG. Plasmapheresis for hemolytic anemia and thrombotic thrombocytopenic purpura complicating pSS is reported. Rituximab has been used as an off-label drug in lymphoma associated with Sjogren's.

Conclusion: Autoimmune cytopenia's may develop before the diagnosis of pSS, and sometimes can be life-threatening. Thus, pSS should be considered in the differential diagnosis of all patients who have otherwise inexplicable cytopenia's.

PM0073: A rare case report of macrophage activation syndrome: A life threatening condition

Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM Medical College, Indore, Madhya Pradesh, India

Macrophage activation syndrome(MAS) is caused by excessive activation and uncontrolled proliferation of T-cells and well-differentiated macrophages which leads to widespread hemo-phagocytosis and cytokine overproduction, a highly stimulated but ineffective inflammatory-immune response, which can be fatal.

Case Report: A 18-year female patient with no significant past and family history presented with easy fatigability, exertional dyspnea of 1-month duration. Low grade fever, cough and nasal bleeds for 5 days.

On examination, pulse rate-100 beats/minute, BP-100/70 mm hg, respiratory rate-18 cycles/minute. On examination pallor was seen with raised JVP. Chest auscultation revealed loud S1 with pan systolic murmur at apex, bibasilar crepitations. On per-abdomen examination hepatosplenomegaly was present. On investigations pancytopenia related to vitamin-b12 deficiency was found and treated with vitamin-b12 supplements, blood and PRP transfusion. Further evaluation showed features of Macrophage activation syndrome with autoimmune thyroiditis and treated with IV methylprednisolone, IVIG, thyroxine therapy and oral immunosuppressants.

Patient deteriorated and developedright upper limb DVT which later complicated as pulmonary thromboembolism.

Bone marrow biopsy-Normoblastic marrow with marked histiocytes and hemophagocytes.

Discussion: MAS is usually seen with systemic juvenile idiopathic arthritis(JIA), still's disease, SLE, Kawasaki disease and characterized by fever, hepatosplenomegaly, generalized lymphadenopathy, CNS dysfunction, and hemorrhagic symptoms. Laboratory abnormalities include pancytopenia, increased levels of ferritin, liver enzymes, LDH, triglycerides, D-dimers, and soluble interleukin-2 (IL-2) receptor and decreased fibrinogen levels. Histopathologic feature of MAS is the accumulation of well-differentiated macrophages exhibiting hemophagocytic activity in bone marrow biopsy specimens or aspirates.

Conclusion: Early recognition and aggressive therapy are critical in MAS. All pancytopenia patients should be evaluated for MAS.

Figure 1: Normoblasticmaturation of bone marrow with many histiocytes (blue arrow), erythrophagocytosis (Yellow arrows), blood vessels proliferatlion (red arrows) and megakaryoacyte (black arrow) Click here to view

Table 1: Lab values of the two cases Click here to view

PM0074: Vasculitis in tuberculosis: A drug or a bug?

R Pratheesh Chandran, M Sudhagar; Pondicherry Institute of Medical Sciences, Puducherry, India

Case Report: A 64-year-old male diagnosed to have active pulmonary TB (Left lower lobe cavitating pneumonia)-sputum positive started on Anti tuberculous therapy. He was started on modified anti tubercular therapy due to alcohol induced hepatitis. His hepatitis resolved in two months. (He was on Isoniazid, Pyrazinamide, Ethambutol and Levofloxacin). Meanwhile patient developed skin rashes-erythema multiforme). Following he was started on steroids and all ATT were stopped (Dechallenged). He was then started with Ethambutol, Amikacin, Levofloxacin. As his hepatitis resolved he was started on Rifampicin and also Pyrazinamide except for Isoniazid (Rechallanged). The skin lesion showed a tremendous improvement after withdrawal of Isoniazid. Hence Isoniazid was not rechallenged. Although a rare entity, Isoniazid among ATT should be considered as a potential cause of Drug induced Vasculitis.

Discussion: Tuberculosis as a disease and also anti tubercular drugs can cause vasculitis. Anti-tubercular drugs induced vasculitis though a known entity Isoniazid induced vasculitis remains rare. This is a case of ANCA positive vasculitis, where drug withdrawal and early initiation of steroids remains the treatment of choice.

Conclusion: With this case report, we aim to create awareness and vigilance about rare, but potentially serious drug reaction. If any ATT induced vasculitis is suspected, Isoniazid as a cause of it should also be considered.

Figure 1 Click here to view

PM0075: Atypical presentations of takayasu arteritis in young females: Case reports

G Mounika Reddy, Bhowmik Meghnathi, Raghurama Reddy; Prathima Institute of Medical Sciences, Karimnagar, Telangana, India

Case Report 1: A 23-Year-oldprimi presented to ER with loss of fetal movements for 2 days. On examination left upper limb pulses were absent, right upper limb B. P 220/110 mm. Hg, left upper limb B. P 110/70 mm. hg, carotid and abdominal bruit heard on auscultation. She was evaluated and investigated further and found to have Intrauterine death of fetus secondary to Takayasu arteritis.

Discussion: TAKAYASU ARTERITIS is commonly associated with first trimester abortions and IUGR. We report a rare occurrence of Intrauterine death with TA.

Case Report 2: A 24-Year-old female known case of TAKAYASU ARTERITIS and on regular follow-up has presented to OPD with redness, watering of right eye for which she was referred to ophthalmology OPD. she was diagnosed with phlyctenular conjunctivitis and was started on antibiotic and steroid eye drops for which patient has not responded. She was further investigated and Mantoux test was positive. She was started on ATT andresponded well.

Discussion: TAKAYASU ARTERITIS have been shown to have association with Tuberculosis with HSP-60kDa as the probable pathogenesis. Phlyctenular conjunctivitis though a common presentation has been rarely presented secondary to tuberculosis. We report a case of phlyctenular conjunctivitis with mantoux positive as a rare presentation of TB, co-existing with TA.

Conclusion: We hereby report 2 cases of Takayasu in young women with varied presentation. Though IUGR and first trimester abortions are noted in Takayasu pregnancy, IUD occurence is rare (<5%). Phlyctenular conjunctivitis though associated with TB, seen as a rare co-association with TA. As there is limited evidence of rare associations of TA, there should be high clinical suspicion and further research has to be done in this field.

Figure 1: Case 2: Phlyctenular coniunctivitis Click here to view

PM0076: Hypokalemic periodic paralysis: A rare presentation of primary Sjogren's syndrome

Ganganpalli Dattaprasad, V P Pandey, Sanjay Dubey; Department of Medicine, MGM Medical College, Indore, Madhya Pradesh, India

Primary Sjogren's syndrome is characterized by dryness of mouth and eyes. Distal RTA is a rare presentation that can cause hypokalemic-periodic paralysis.

Case Report: A 33-year female with no significant past and family history admitted with progressive quadriplegia for four days.

On examination, pulse rate was 78 beats/ min, Blood pressure 110/70 mm of hg, Respiratory rate 16 cycles/ minute. Motor examination revealed flaccid quadriplegia with power 2/5 in all four limbs with sluggish reflexes and flexor plantars. ECG showed prominent U waves. ABG was suggestive of normal anion gap metabolic acidosis with pH 7. 085. LFT, RFT, and TFT were normal. NCS ruled out AIDP.

Retrospective history revealed dryness of eyes and mouth with foreign body sensation in eyes since past few years. Treated with intravenous potassium chloride and the patient improved.

Discussion: Distal RTA characterized by an inability of the distal nephron to acidify the urine leading to hyperchloremic, hypokalemic, metabolic acidosis with a normal serum anion gap and urine pH greater than 5. 5.

The common cause of distal RTA is diminished H+-ATPase activity with resultant hypokalemia leading to urine pH persistently >5. 5. The most common histological renal lesion in RTA with Sjogren's syndrome is interstitial nephritis characterized by lymphocytic and plasma cell infiltrates surrounding renal tubules and by hypergammaglobulinemia leading to distal renal tubular dysfunction and loss of H+-ATPase pumps in the intercalated cells. Steroids are useful in refractory cases.

Conclusion: All acute flaccid paralysis patients should be looked for autoimmune causes. < 5% patients with Sjogren syndrome present with hypokalemic periodic paralysis and distal RTA.

Figure 1: ECG showing features of hypokalemia Click here to view

Table 1: Investigation details Click here to view

PM0077: Rarer presentation of a rare disease in pediatric population

S Rajesh , G Asok Kumar, Anu K Vasu; Kerala Institute of Medical Sciences, Thiruvanathapuram, Kerala, India

A 13-year-old boy was admitted with history of fever for 2 weeks with erythema over lips and tongue with no lymphadenopathy. Blood investigations were normal except for high ESR 65mm/hour and CRP 31mg/L. Echocardiogram was normal, chest X ray showed haziness over the lower zone of the left upper lobe and hence treated with antibiotics for 7 days with resolution of fever. On follow up at 6 weeks, patient was asymptomatic but had persistent high ESR and CRP. Ultrasound showed mild hepatosplenomegaly and echocardiogram revealed thickened posterior pericardium with minimal pericardial effusion. HRCT chest showed multiple mediastinal lymphadenopathy, consolidation of left upper lobe and an anterior mediastinal mass probably thymic mass. Repeat echocardiograph done at 10th week showed coronary artery aneurysm, pericardial effusion, thickening around proximal aorta suggestive of aortitis and mild impairment of LV function. 18F-FDG PET/CT study revealed metabolically active mediastinal adenopathy, thymic and pericardial infiltrates and a metabolically active right iliac bone lesion. Mediastinoscopic lymph node biopsy was suggestive of benign reactive lymph node with plasmacytosis. On immunohistochemistry an increase in IgG4 positive plasma cells were identified. Ig G4 positive plasma cell count per high power field was 30-35. The ratio of IgG4 positive to CD 138 positive plasma cells was 35-40%. Serum Ig G4 level was elevated, 292mg/dl. Treatment was initiated with steroids tapered gradually with normalization of inflammatory parameters and repeat cardiac MRI showed normal sized coronaries.

Discussion: This case presenting with fever and then during immediate follow up evaluation revealing a rarer cardiac involvement in the form of coronary artery aneurysm with confirmatory histopathological features of IgG 4 disease. IgG4-related cardiac manifestations include involvement of the myocardium, 3rd degree heart block due to SA node involvement, cardiomegaly, constrictive pericarditis, pseudotumours around coronary arteries, valvular regurgitant lesions involving the aortic and mitral valves.

PM0078: Thionamide induced myositis- report of a case and systematic review of literature

Koshy Nithin Thomas, Sujata Ganguly, Durga Prasanna Misra, Ramnath Misra, Latika Gupta; Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, Uttar Pradesh, India

Discussion: A 31-year-old lady presented with rashes and muscle weakness 2 weeks after taking Carbimazole for Grave's disease. She had Gottron's papules and a Heliotrope rash with proximal myopathy. Investigations revealed elevated muscle enzyme levels, positive ANA and an abnormal EMG. Unlike most earlier case reports of Thionamide induced myositis where cessation of the drug has resulted in resolution of symptoms, her muscle weakness continued to worsen. She was given I131 therapy for her Grave's disease, and her muscle weakness and rashes gradually improved with immunosuppressive therapy over six months.

Conclusion: Though rashes are a well described adverse effect of Thionamides, the development of a rash in the setting of muscular symptoms is not common in literature (n=13). Rarely, there have been case reports of Anti-thyroid drug induced lupus^[1] as well as ANCA vasculitis.^[2]. An “antithyroid arthritis syndrome”, which comprises of fever, rash, pruritus, arthralgia and myalgia has been reported. However, muscle weakness in not a feature of this syndrome.^[3] The development of frank dermatomyositis induced by Thionamides has not been previously reported in adults.

References

1. Wang LC, Tsai WY, Yang YH, Chiang BL. Methimazole-induced lupus erythematosus: A case report. J Microbiol Immunol Infect 2003;36:278-81.
2. Gunton JE, Stiel J, Caterson RJ, McElduff A. Clinical case seminar: Anti-thyroid drugs and antineutrophil cytoplasmic antibody positive vasculitis. A case report and review of the literature. J Clin Endocrinol Metab 1999;84:13-6.
3. Shabtai R, Shapiro MS, Orenstein D, Taragan R, Shenkman L. The antithyroid arthritis syndrome reviewed. Arthritis Rheum 1984;27:227-9.

PM0079: Rare case of coexisting polyarteritis nodosa and antiphospholipid syndrome: A diagnostic and therapeutic challenge

W A E Udeshika, R M Wickramarachchi, M De Silva^1,2Senior Registrar in Rheumatology,³Consultant Rheumatologist

Case: A 50-year-old man with hypertension and type 2 diabetes mellitus with macro vascular complications, 6 months ago presented with high grade fever, constitutional symptoms, bilateral upper and lower limb rash with reduced conscious level for 3-weeks duration.

On examination he had a vasculitic rash in upper and lower limbs, dry gangrene in left second and third toes with impalpable peripheral pulsation. Further he had distal, motor sensory peripheral neuropathy and reduced consciousness with GCS 12/15 with no signs of meningism or focal neurological signs.

Hematological investigations revealed neutrophil leukocytosis, anemia, mild thrombocytopenia, and elevated ESR and CRP. Septic screening, HIV, ANA, ANCA, serum cryoglobulins were negative. MRI brain, MRA, MRV revealed acute infarctions in left parietal lobe with small vessel disease. Skin biopsy revealed medium vessel vasculitis with fibrinoid necrosis and CT mesenteric angiogram revealed vasculitic changes in mesenteric vessels. He was treated with IV methyl prednisolone and had a dramatic response. Ward stay was complicated with extensive thrombosis in pelvic veins. His antibodies for Lupus anticoagulant became positive, 12 weeks apart and started on lifelong warfarin therapy for Antiphospholipid syndrome. Despite aggressive immunosuppression with cyclophosphamide, he remained bed bound with high morbidity.

Discussion: This patient with evidence of medium vessel vasculitis as evident by fibrinoid necrosis in the skin biopsy with mesenteric and neurological manifestations warrants treatment with aggressive immunosuppression. Coexisting stroke and extensive thrombosis in the pelvic veins raised a therapeutic challenge of starting anticoagulation given after weighing benefits over risks.

Conclusion: Antiphospholipid syndrome(APLS) secondary to systemic vasculitis is extremely rare, reported roughly 20 cases in literature. This patient who had biopsy proven Polyarteritis nodosa had unusual presentation with mild thrombocytopenia, extensive pelvic and abdominal vein thrombosis and positive lupus anticoagulant assay, which can only be explained by coexisting APLS.

PM0080: Retinal vasculopathy in children with systemic lupus erythematosus: Report of 2 cases

Sandesh Guleria, Ankur Jindal, Dharmagat Bhattarai, Anirudha Aggarwal, Bhartendu Sharma, Ramandeep Singh, Deepti Suri, Surjit Singh; Postgraduate Institute of Medical Education and Research, Chandigarh, India

Case Description: Patient 1: A 9-year-old boy presented with fever and vomiting for 15 days. Examination was unremarkable except for pallor. Laboratory investigations revealed anemia and thrombocytopenia.

During fundus examination, he was found to have cotton wool spots in both eyes. He was further worked up for lupus. ANA (4+ speckled pattern) and anti-dsDNA were positive. Fundus FFA showed capillary 'drop-out' and leakage of dye. These findings were suggestive of lupus retinitis. His refraction testing revealed low vision in both eyes (perception of light at 3 feet).

He was given 5 daily pulse of injection methylprednisolone followed by oral prednisolone. Six, monthly injection cyclophosphamide (500 mg/m2/month) were also administered followed by azathioprine. He was also initiated on hydroxychloroquine, warfarin and aspirin. He had gradual improvement in vision and at 1½ years of follow up, his vision has improved to a visual acuity of 6/36 in both eyes. Fundus examination showed significant reduction in cotton wool spots and repeat FFA has revealed no leakage.

Patient 2: An 8-year-old boy presented with fever and rash for 1 month. Examination revealed malar rash and oral mucosal ulcers. Laboratory investigations showed lymphopenia, positive ANA and anti-dsDNA. His fundus evaluation showed cotton wool spots. FFA was suggestive of capillary 'drop-out' and leakage of dye. Visual acuity was normal.

He was given 5 daily pulse doses of injection methylprednisolone followed by oral prednisolone. Six monthly pulse doses of injection cyclophosphamide were also administered. He was later put on mycophenolate mofetil. At 1. 5 years of follow up, he has quiescent disease with normal fundus examination.

Discussion and Conclusion: Retinal vasculitis is a serious ocular manifestation of lupus. All children with lupus should have detailed ophthalmological evaluation for this potential complication. Children with vaso-occlusive retinopathy need aggressive immunosuppressive therapy and the prognosis remains guarded.

PM0081: Pancreatitis in SLE

N Rishabh, C Balakrishnan, Y Sandeep, S Sameer; PD Hinduja Hospital and Research Center, Mumbai, Maharashtra, India

SLE is a disease with protean manifestations. Pancreatitis though rare does occur and has been thought as a complication of drug treatment rather than disease. However recent reports including onset of lupus with pancreatitis suggest that this may be manifestation of an autoimmune process.

We have come across 5 patients with lupus pancreatitis.

All patients were females in second and third decade of life and presented with abdominal pain and vomiting.

Labs showed low C3 and raised amylase and lipase and positive ANA and dsDNA and imaging showed bulky diffuse pancreatitis. All patients were treated with steroids and use of steroid sparer.

Discussion: Lupus Pancreatitis is a rare entity with incidence of 0. 4-1. 1/1000 cases of SLE.

Various mechanism includes vasculitis microthrombi and intimal thickening.

Carries high mortality rate of 27% higher than non SLE associated pancreatitis.

Although uncommon with lupus suspect pancreatitis if a patient presents with vomiting abdominal pain and the need to rule out other causes. Pancreatitis may be an initial manifestation hence early recognition is vital since it is associated with high mortality rates.

PM0082: Gouty arthritis in a premenopausal woman: An unusual case

Suraj Chaudhari, Lubna Khurshid, Sanjiv Kapoor, Anand N Malaviya; Department of Rheumatology, ISIC Superspecialty Hospital, Vasant Kunj, New Delhi, India

Case Report: A 54 year old woman presented for the first time with 8 days of acute inflammatory arthritis of the knees, ankles, right elbow, and low-grade fever for 2 days. She had intermittent, asymmetrical arthritis involving large and small joints for the past 20 years; treated as seronegative RA with glucocorticoids and NSAIDs with transient relief and repeated flares in different joints. Her vital signs were normal, Cushingoid face, swollen and tender knees, ankles and the right elbow with subcutaneous nodules and gross deformities in several hand joints with wasting of quadriceps. Hand radiograph showed damage and deformities]. The examination of the other systems was unremarkable.

Investigations: High ESR 117 mm, Hb-8. 9 gm/dL, WBC 7490/cmm, platelets 1, 52, 000 /cumm. Serum Creatinine - 1. 02 mg/dL, ALT/AST: 19/34 units/ml, serum uric acid 12. 4 mg/dL, Urine R/M showed monosodium urate crystals, RF and ACPA -negative. The right knee joint synovial fluid showed negatively birefringent MSU crystals seen in polarised light microscopy. Test hypoxanthine-guanine phosphoribosyltransferase (HGPRT) and 5-phosphoribosyl-1-pyrophosphate (PRPP) enzyme could not be done.

Discussion: Gouty arthritis is mainly a disease of males > 40 years of age. Therefore, it considered most unusual in women in reproductive age except if they: (i) Have deficiency HGPRT or increased levels of PRPP enzyme; (ii) Are on long-term cyclosporine treatment (iii) Have chronic kidney disease. Diagnosis is based upon clinical characteristics and demonstration of MSU crystals in synovial fluid. Targeted treatment with SUA to be kept < 5 mg/dL over long-term is effective.

Conclusion: Gouty arthritis should be considered as one of the possibilities in an asymmetrical seronegative oligo-polyarthritis with acute intermittent attacks. Presence of subcutaneous nodules may also suggest the possibility of gouty tophi. Such patients must be carefully investigated for gouty arthritis because it is a treatable condition.

PM0083: Connective tissue diseases with rare associations: Nightmare for rheumatologists

Vaijayanti Joshi, SmitaUpadhye, Sameer Melinkari, Sameer Jog, Sujit Jagtap; Deenanath Mangeshkar Hospital and Research Centre, Pune, Maharashtra, India

Background: Managementofconnective tissue disorders(CTD)likesystemic lupus erythematosis (SLE), sclerodermais always achallenge for rheumatologists. Therearevaried multisystemic manifestationssometimes CTDs show rare associations with macrophage activation syndrome(MAS), thrombotic thrombocytopenic purpura(TTP), neuromyotoniaand become a nightmare for management.

Objective: Toshare experience of 3 cases, 2 of SLE, one presented with TTP, 1 withMAS. 1 case of scleroderma presented withneuromyotonia. To discuss management of the conditions.

Methods: CASE 1:39 YR female known SLEonHCQS, leflunomide for her arthritis presented with generalized weakness, anemia and jaundice, initially thought of leflunomide toxicity but after evaluation turned out to have TTP. She underwent plasmapheresis and rituximab infusions and showed a significant recovery.

Case 2: 26yr male known case of SLE presented with high grade fever, cytopenia and splenomegaly. He was extensivelyworked up for infections finallywas diagnosed to have MASsecondary to SLE and treated with high dose steroids and rituximab. He responded dramatically. He remained in remission for 3 years however showed a relapse and managed on same line.

Case 3: 44yr male diagnosed as systemic sclerosis presented with severe backache and myalgia followed by spontaneous persistent muscle twitching of calves, thighs and arms. He developed severe insomnia and restlessness. EMG confirmed it to be neuromyotonia and he had voltage gated potassium channel(VGKC) antibodies. He responded well to plasma exchanges and immunosuppression.

Results and Conclusions: The literature shows few case reports and series sharing CTDs with those rare associations. Although etiologies are elusive certain autoimmune mechanisms may be shared and provide basis for their associations.

Timely diagnosis and aggressive treatment are needed as they can be life threatening.

Keywords: SLE, TTP, MAS, neuromyotonia, scleroderma

PM0084: Acute liver failure in a patient with SLE and autoimmune hepatitis

Anjana G Varier, C E Eapen, Philip Joseph, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Case Report: A 30 year old female, a case of SLE with autoimmune haemolytic anemia diagnosed 10 years back presented to us with history of jaundice and vomiting of 2 weeks duration. On evaluation she was found to have acute severe hepatitis. She was diagnosed with autoimmune hepatitis after excluding infection, drug induced liver injury and Budd Chiari syndrome. She was started on steroids but her disease rapidly worsened to fulminant hepatic failure. A decision for urgent liver transplantation was made after multidisciplinary discussion. An orthotopic liver transplantation was done following which she recovered completely. Her disease activity is currently well controlled on the immunosuppression for liver transplantation and she is asymptomatic since then.

Discussion: Co-occurrence of SLE and Auto Immune Hepatitis is rare. It is also rare for autoimmune hepatitis to result in acute liver failure. The important issues in management of Acute Liver Failure by Autoimmune Hepatitis are: (A) establishing an appropriate diagnosis; (B) evaluation of risks and potential benefits of immunosuppressive therapy; and (C) urgent consideration for Liver Transplantation. Emergency Liver Transplantation becomes crucial if responses toimmunosuppressants are not confirmed promptly after introduction. We report a case of auto immune hepatitis who presented to us 5 years back who had rapidly worsened to fulminant hepatic failure. Emergency intervention in the form of liver transplantation within 10 days prevented the patient from succumbing to her disease. The ongoing immunosuppression for transplant has kept her clinically and serologically quiescent for the autoimmune disease.

Conclusion: Prompt diagnosis of autoimmune hepatitis and early evaluation of its response to immunosuppressants with early decision for liver transplantation can improve the mortality and morbidity of these patients.

PM0085: Osteomalacia: Mimicking the myopathy

Sandeep Nagar, Debaditya Roy, Uma Kumar; Department of Rheumatology, AIIMS, Delhi, India

Case Report: A 16 years girl presented with history of pain in both upper limb for 1. 5 years, started proximally then progressed to distal associated with weakness. She was having bilateral chest pain for 6 months, pain in both arms and back pain for 1 month. Pain was persistant on rest and activities, intensity 10/10 (VAS), with nocturnal awakening, butock pain, difficulty in sitting and squatting. this pain used to relieve with analgesics. She had history of seizure disorder at the age of 9 for which she received valproic acid for 6 months.

On examination her gate was antalgic, tenderness over chest wall. muscle power was 5/5 after alleviating pain. She had no history suggestive of any rheumatic or neurological disease.

Her hemogram, liver and kidney functions were normal, with normal electrolytes, thyroid profile, vit D, B12, folic acid. Viral markers, ENA profile ESR, CRP, CPK, LDH, acid alpha glucosidase, NCV, EMG all were normal. serum total Ca was 9. 6 mg/dl, PO4-3. 4 mg/dl, ALP-146 IU/L. Her xraypeviswasdone which showed looser zones and hazy sacroiliac joint margins.

On the basis of clinical features and radiological findings diagosis of osteomalacia was made then she was started on calcium and vit D supplementation (RDA) and analgesics.

Figure 1 Click here to view

Discussion: Osteomalacia means soft bones. It occurs when there is insufficient mineral or osteoblast dysfunction so that osteoid does not mineralize properly. Decreased vit D is the most common cause of osteomalacia, some inherited conditions that affect mineralization can also be responsible. Usually symptoms and blood tests are enough to make the diagnosis but sometimes x-ray can show up the changes.

Conclusion: Presenting this case of oasteomalacia in which diagnosis was delayed in suspicion of neuromuscular or rheumatologic disorders.

PM0086: IgG4 related disease with tubulointerstitial nephritis: A case report

Daisy Doley, Mandeep Bindra, Jennifer Prabhu, John Mathew; Christian Medical College, Vellore, Tamil Nadu, India

Background: IgG4- related disease (IgG4-RD) is an immune mediated, fibroinflammatory condition with multisystem involvement. It is characterised by storiform fibrosis, obliterative phlebitis and dense lymphoplasmacytic infiltrate of affected organs on biopsy. IgG4-tubulointerstitial nephritis (IgG4-TIN) is the major manifestation of renal involvement in IgG4-RD, leading to acute or chronic kidney dysfunction. The mainstay of treatment is corticosteroids.

Case Presentation: We describe here a 53 year old gentleman diagnosed as a case of chronic kidney disease for 1 year who presented with bilateral parotid involvement and submandibular swelling for which he was referred. Renal biopsy demonstrated plasma cell rich interstitial inflammation with eosinophils in tubulointerstitial compartment. Immunofluorescence for IgG showed tubular basement membrane, interstitial and Bowman capsule granular staining. IgG4 staining shows 35 IgG4(+) cells/40x field. IgG4/IgG(+) plasma cell ratio was 30%. Biopsy from left parotid gland showed dense lymphoplasmacytic infiltrate with patchy fibrosis consistent with a probable diagnosis of IgG4 relate disease.

Discussion: The hallmark of IgG4-RD is dense lymphoplasmacytic infiltration rich in IgG4 secreting plasma cells, resulting in storiform fibrosis and obliterative phlebitis. Similar lesions have also been described in other organs with varying manifestations. TIN is the most common renal manifestation of IgG4 related renal disease. The pathogenesis of IgG4-RDremains poorly understood. Differential diagnoses of IgG4-RD include malignancy, connective tissue diseases, vasculitisand other infiltrative diseases. Corticosteroids are the first line of treatment and response is dramatic. we intend to put the HPE pictures of both biopsies andIgG4 plasma cell staining in the presentation.

Conclusion: Patient was initiated on steroid therapy and showed good clinical response to therapy. He has been kept on follow up. IgG4-TIN must be kept as a differential diagnosis in elderly patients prrsenting with renal dysfunction.

Keywords: IgG4-related disease, Tubulointerstitial nephritis, Parotid involvement, Corticosteroids

PM0087: Acute lupus pneumonitis: Atypical presentation of prototypical disease

Ankur Dalal; The Sarvajanik Medical Trust Hospital, Surat, Gujarat, India

Case Report: A 21 year female received on non-invasive ventilator support with provisional diagnosis of acute viral haemorrhagic fever probably dengue due to short history of high fever, headache, arthralgia, skin rash, thrombocytopenia, elevated transaminases for 1 week and acute respiratory failure (bilateral patchy-inhomogeneous perihilar+lower-zone opacities on chest x-ray-Extensive pneumonitis/ARDS, PO2-60mmHg) for 1 day. Patient had history of blood transfusion (haemoglobin-7. 2gm %) before admission. On investigations; WBC, creatinine, procalcitonin, LDH, CPK, Dengue duo, S. Widal, HIV/HBsAG/HCV, coomb's test, DIC profile, USG abdomen-pelvis, and 2D-echo were normal. Due to breathlessness, anemia, thrombocytopenia (1. 13 Lac/cumm), very high ESR (90 mm/hr), and normal CRP the patient was investigated by keeping in mind differentials like acute lung injury (ALI) secondary to viral or atypical bacterial infection, transfusion related ALI, secondary haemophagocyticlymphohistiocytosis, systemic autoimmune connective tissue diseases (AICTD) and systemic vasculitis. However, further work-up ultimately lead to diagnosis of systemic lupus erythematosus (SLE) presented with acute lupus pneumonitis (LP) with subclinical neurological involvement.

Investigation Results: Urine protein0. 48 gm/24-hr Sputum/Blood culture, Ferritin, ANCANegative HRCT-lungBilateral perihilar patchyconsolidation/minimal pleuraleffusion-Atypicalpneumonitis/ARDS MRI-brainDemyelination/Encephalitis C3/C4Low/Normal ANASpeckled+3 ANA-profileAnti-Sm+3/Anti-RNPsm+3/Anti-dsDNA+3/Anti-Nucleosome+3/Anti-histone+2 Patient was started on methylprednisolone and cyclophosphamide pulse therapy and responded dramatically (her hypoxemia and chest x-ray were normalized within 3 days). Patient was discharge on oral prednisolone 1mg/kg in tapering schedule and hydroxychloroquine. Later azathioprine was added as steroid sparing.

Discussion: Acute LP is a rare manifestation of SLE with high mortality and can be very difficult to distinguish from other differentials (especially infection) on first presentation at disease onset. Early diagnosis and treatment with steroids are essential for better outcome.

Conclusion: High index of suspicion of LP, timely investigations, and early treatment is the key for favorable prognosis in patients presented with this atypical lethal manifestation of prototypical AICTD - SLE.

PM0088: Amyloidosis in rheumatoid arthritis: A learning experience

Kattel Vivek, Riyaz Asad, Malviya Sourabh; Medanta Superspecialty Hospital, Indore, Madhya Pradesh, India

Introduction: A amyloidosis is a disorder characterized by the extracellular tissue deposition of fibrils (serum amyloid A protein). Amyloidosis in inflammatory arthritis is associated with increase morbidity and early mortality.

Case Summary: A 58 years female known case of seropositive Rheumatoid arthritis under three DMARDS presented with sudden rise in creatinine (1. 7 mg/dl to 3. 06mg/dl), hematuria, granular cast and albuminuria (1. 27gm/24hours). On evaluation her blood pressure was 140/90mm/Hg with 7 tender joints and 3 swollen joints. Besides significant blood investigations were raised ESR (100mm/hr) and CRP (10mg/dl), reversal of serum protein (Serum albumin 3. 2g/dl, serum globulin 4. 2g/dl) and anemia (Hb 9. 2gm/dl). In view of rapid rising creatinine renal biopsy was secured favoring diagnosis of crescent nephritis. Biopsy revealed amyloid deposit in glomeruli and vascular region with acute patchy injury over chronic tubule-interstitial changes. She was treated with rituximab induction dose followed by two six monthly doses. She has improvement on creatinine (1. 5mg/dl) and urine (microscopic examination protein trace).

Discussion: Risk of developing amyloidosis in RA is related to duration (mean duration 15. 4years), high disease activity, seropositive, extraarticular manifestations and poorly controlled rheumatoid. RA is common cause of AA Amyloidosis predominantly involving kidneys followed by gastrointestinal system and cardiomyopathy. Renal manifestation depends upon deposit site (vessels, glomerulus and interstitial). Glomerular involvement in form of massive proteinuria and rising creatinine is common clinical presentation among more than 90% cases. Tissue biopsy is diagnostic test. One forth to half of subclinical amyloid fat deposit on abdominal biopsy only develop clinical amyloidosis. Among asymptomatic joints RA high ESR and CRP can be clue of amyloidosis. Biological DMARDs (Rituximab) can be promising to control the primary disease and renal morbidity.

Conclusion: Biological DMARDs have better outcome with secondary amyloidosis in RA.

Keywords: Amyloidosis, rheumatoid arthritis, rituximab

PM0089: A case of limited cutaneous systemic sclerosis with decompensated chronic liver disease

Karthik Harisankar, N G Taruni Devi, N Santa, K Romeo Singh; Regional Institute of Medical Sciences, Imphal, Manipur, India

Case Report: 55 year old female came with complaints of fatigue, occasional pruritus and typical salt and pepper pigmentationin the anterior aspect of forehead and anterior part of leg. She also had abdominal distension with history of constipation and was drowsy at presentation. She had history of hypothyroidism on medication and was recently detected to be diabetic. LFT showed raised bilirubin and alkaline phosphatase. ANA was positive with 1 : 1280 endpoint titre. ENA screen showed positive anti centromere protein B. Skin biopsy was confirmatory of scleroderma. USG showed coarse liver echotexture with ascites. Viral markers were negative. Autoimmune hepatitis panel showed a positive Antimitochondrial antibody and rest of the antibodies were negative. A diagnosis of limited cutaneossysytemic sclerosis with decompensated chronic liver disease with ascites and hepatic encephalopathy was made. Etiology of chronic liver disease was attributed to Primary biliary cirrhosis in view of raisedALP, positive AMA and after exclusion of other causes of chronic liver disease. The possibility of a backdrop of NAFLD was also considered. Patient was managed with immunosuppresants and ursodeoxycholic acid.

Table 1: Laboratary parameters Click here to view

Discussion: In systemic sclerosis, liver is rarely affected but occurrence of primary biliary cirrhosis is found to coexist in approximately 2 percent cases. This association is called as Reynolds syndrome. Pateints with Scleroderma and chronic liver disease are prone to Hepatic encephalopathy due to bowel dysmotility and bacterial overgrowth.

Conclusion: Work up for liver disease also should be undertaken in a case of systemic sclerosis and the possibility of coexisting primary biliary cirrhosis has to be considered.

PM0090: Congenital insensitivity to pain with anhydrosid

Anuja Sapre; Kalawati Saran Children Hospital, New Delhi, India

Background: CIPA(congenital insensitivity to pain with anhydrosis) is charaterized by insensitivity to pain, inability to sweat, self mutilatingbehaviour and mental retardation. It is very rare.

Case Presentation: 8 year old with complaints of recurrent boils and pustules and recurrent osteomyelitis that required above knee amputation of right lower limb. The child was DHR positive. Physical examination revealed no withdrawl to pain stimulus. His parents were cousins. There was death of a younger sibling with similar complaints at 3 years of age. The diagnosis of CIPA was confirmed on biopsy.

Conclusion: No cure is available. Preventive approach remains the only possible treatment. Early diagnosis and specific management could result in reduction of frequency and severity of complications.

PM0091: Polymyxin B induced skin hyperpigmentation in a SLE patien

Sarath Jakka, Pradeep Kumar; Narayana Health City, Bengaluru, Karnataka, India

A 40 year old man with history of hypothyroidism presented with the complaints of breathlessness and hemoptysis and it was diagnosed to be due to chronic pulmonary thromboembolic pulmonary hypertension. His blood tests revealed anti phospholipid (APLA) syndrome with positive anticardiolipin antibodies IgM and IgG. Direct Coombs test was also positive. ANA (IF) was negative but complement C4 was low. He then developed abdominal pain with CT scan showing features of bowel vasculitis in jejunum, terminal ileum and cecum. Provisional diagnosis of SLE was done with secondary APLA and bowel vasculitis. He responded well to IV methyl prednisolone and maintained on oral prednisolone and hydroxychloroquine. He then underwent pulmonary thromboembolectomy successfully but the post-operative period was complicated by pneumonia with gram negative MDR Klabsiella septicemia sensitive to polymyxin B and Colistin. Polymyxin B was chosen to be given for 14 days at the dose of 7. 5 lakh units twice a day. Within a week after starting antibiotics, his skin colour darkened dramatically and it persisted for more than 2 months after. We stopped his hydroxychloroquine and maintained on low dose prednisolone and warfarin. His general condition was stable and once his INR is stablised we will be introducing mycophenolate mofetil.

Learning Point: Patients with SLE who already are prone for skin photosensitivity and also taking hydroxychloroquine which also slowly causes skin hyperpigmentation, should avoid polymyxin b injection if a suitable alternative is available.

PM0092: Granulomatosis with polyangiitis presenting as gastric outlet obstruction: A case report

S Bhatt, V Vasdev, Ramakant, A Kumar; AHRR, Delhi, India

A 32-year-old lady presented with 4 months' history of insidious onset epigastric pain, vomiting and abdominal distension, aggravated by meals. She also developed intermittent fever, dry cough and significant weight loss (14 Kg over last 4 months). She denied any history of gastrointestinal bleeding, hemoptysis, breathlessness or ENT manifestations. Evaluation revealed normocytic normochromic anemia (Hb 8. 9 g/dl), normal renal function and urine analysis. Chest imaging showed presence of cavitatory pneumonia in bilateral lungs and endoscopy revealed presence of ulcero-proliferative growth at GE junction. Histopathology of the lesion showed polypoidal hyperplasia with mixed inflammatory infiltrate in lamina propria and mucosa with absence of atypia or malignant cells. Work up for chronic infective and malignant etiology, including malignancy markers and bone marrow study was negative. Further evaluation revealed c-ANCA positive with elevated anti-PR3 titres. She underwent CT guided FNAC from lung lesion which revealed presence of non-caseating necrotizing granulomas. She has been managed with intravenous pulse methyl prednisolone followed by eight Cyclophosphamide pulses as per EUVAS regimen with good response to treatment. She has remained afebrile with improvement in gastrointestinal symptoms, weight gain and significant reduction in the size of gastric mass and outlet obstruction on follow-up endoscopy.

Discussion: GPA is a systemic disease characterized by necrotizing granulomatous inflammation and vasculitis that primarily involve upper and lower respiratory tracts, as well as kidneys. GI involvement in GPA is uncommon and outlet obstruction mimicking gastric malignancy is extremely rare with only few cases reported in literature. Although the biopsy did not reveal presence of granuloma, our patient had raised PR3 titres with evidence of necrotizing granulomas in lung FNAC.

Conclusion: Although rare, GI involvement may be a presenting feature in GPA, with occasional occurrence as gastric tumor-like lesions. Diagnosis should be considered in patients presenting with GI symptoms accompanied by evidence of systemic vasculitis.

PM0093: Kawasaki disease presenting with peripheral gangrene

Deonath Mahto, Anu Maheshwari, Rohit Duggar; Department of Pediatrics, Division of Rheumatology and Immunology, Lady Hardinge Medical College and Associated Kalawati Saran Children's Hospital, New Delhi, India

Kawasaki disease is the most common cause of acquired heart disease in childhood. Early diagnosis and treatment may significantly improve the prognosis. Diagnosing Kawasaki disease in younger children is difficult because of obscure and atypical symptoms, moreover they are at the higher risk of coronary artery abnormalities and complications.

We report a 14 month old baby with fever of 10 days duration and peripheral gangrene without any other clinical manifestations of Kawasaki disease. Kawasaki was diagnosed subsequently due to mild dilation(LMCA SD score +1. 89, LAD SD score +2. 28) of coronary artery and lack of tapering of the arteries on Echo cardiography. After 1 month of follow up, reduction of LMCA SD Score to +0. 77 was confirmatory as per 2017 AHA criteria. In addition, on follow up he developed cracking of lips, periungal and perianal desquamation. He also had history of red eyes early in the course of disease which was absent at the time of presentation. He was treated with high dose aspirin, IVIG and pulse therapy with methylprednisolone. He received aspirin and warfarin on follow up.

To conclude, Peripheral gangrene must be regarded as an important sign of Kawasaki disease in younger children and early treatment can prevent severe permanent coronary involvements and sequae.

PM0094: An unusual presentation of medium vessel vasculitis

M Sabarinath, T N Tamilselvam, N Balakrishnan, H Raghavendra, Anusha Sri Matam, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

27 year male presented with fever, polyarthralgia and painful bullous skin lesions in lower limb for 2 weeks duration. On eliciting past historypatient had multiple ulcers in legsfollowing similar skin lesions andgangrene of finger tips at 8 yrs of age. He was treated as vasculitis with cyclophosphamide 6 doses and maintained on MMF. The current skin lesions appeared after he defaulted treatmentfor 4 months. He has no other comorbid illness including claudication pain or smoking. On examination patient had tenderbullous lesionsand induration involving both lower limb with multiple papery scars in legs. He also had amputation of his left 5th digit, resorption of right 2nd digit and residual hyperpigmentation over abdomen, limbs. Rest of his examination was normal and all his peripheral pulses were palpable with no bruit in neck, abdomen. On evaluation his complete blood counts were normal with elevated ESR and CRP. His renal, liver function were normal and viral markers were negative. His immunological workup was negative for RF, ANCA, ANA and cryoglobulins. His skin biopsy showed supra basal bulla with inflammatory infiltrate of predominantly mononuclear cells around dermal appendages and engorged vessels suggestive of vasculitis. His CT angiography showednear total occlusion of right superficial femoral, anterior tibialsuggestive of peripheral vascular disease with normal upper limb, abdominal vessels. His APS and prothrombotic workup was negative. He was treated with pulse steroids, MMF, aspirin, supportive measures. His bullous lesions subsided with peeling of skin and ulcers healed with residual hyperpigmentation. We present this as a case of childhood PAN with cutaneous lesions, gangrene in fingers with rare association of total occlusion of lower limb vessels.

Figure 1: Skin lesion showing tender nodules with bullous lesion, CT angio showing occulusion of right femoral artery Click here to view

PM0095: Multifaceted and intriguing presentations of male scleroderma patients: A case series from clinicians perspective

N Balakrishnan, N Sabarinath, T N Tamilselvam, R Ramesh, S Mythili; Department of Rheumatology, Madras Medical College, Chennai, Tamil Nadu, India

Case 1: 54/M presented with breathelssness for one month. He was diagnsosed as pulmonary thromboembolismbased on echo and CTPA. He was treated with anticoagulation. During evaluation he was found to have sclerodactyly, Raynauds, salt and pepper pigmentation over scalp and thyromegaly. His ANA status revealed nucleolar pattern by IIF. There was no evidence of ILD and GIT manifestation. Hypercoagulable workup was negative. He was treated with low dose steroid and Azathioprine.

Figure 1: Salt and pepper pigmentation Click here to view

Figure 2: HRCT chest showing ILD Click here to view

Figure 3: Sclerodactyly Click here to view

Case 2: 47/M presented with recurrent episodes of AGE, chronic anemia requiring blood transfusion, loss of weight and appetite since 2010. In 2014 he developed inflammatory polyarthritis and frequent AGE episodes. Anemia was persisting. OGDscopy revealed distal esophagitis and pangastritis. He was treated conservatively. In 2016 he was admitted in our centre with limited cutaneous scleroderma and ILD based on clinical features and ANA positivity. The cause for anemia was found to be AIHA based on DCT positivity. ANA testing revealed speckled pattern by IIF. He was treated with steroids and immunosuppression and he is under follow up.

Case 3: 40/M presented with shortness of breath for two months duration in 2017. Echo revealed moderate pulmonary hypertension without RA, RV dilatation. Three months later he was diagnosed aslimited cutaneous scleroderma and ILD with NSIP pattern. He presented to our centre with inflammatory polyarthritis and GERD. He had arthralgia of large and small joints, sclerodactyly, salt and pepper pigmentation, raynauds with pitted scars on clinical examination. ANA status revealed homogenous pattern by IIF. He was incidentally found to be positive for hepatitis B antigen. He was being treated with calcium channel blockers and phosphodiestrase 5 inhibitors.

We present this case series of multifacted presentation of male scelroderma patientswith initial manifestationofPulmonary embolism, Anemia and inflammatory polyarthritis, Pulmonary hypertension.

PM0096: Pancreatitis-panniculitis-polyarthritis syndrome with plexopathy

Rakesh Kumar Jagdish, Ankur Jindal; Department of Hepatology, ILBS, Delhi, India

Pancreatitis-Panniculitis-Polyarthritis (PPP) syndrome is rare extrapancreatic complication of pancreatic disease with unclear physiopathology. There are few case reports in literature of a syndrome consisting of pancreatitis, panniculitis and polyarthritis known as 'PPP syndrome'. We report a patient with 4th “P” plexopathy along with this “P 3” syndrome. There is only scanty information in literature regarding this type of presentation. A case of38 year old male patient without any prior co morbidities, presents with recurrent mild acute pancreatitis (4-5 episodes) over last 1. 5 years. During the first episode of pancreatitis, he developed symmetrical polyarthritis (swelling of bilateral wrist, bilateral knee and bilateral ankle joint), subsequently leading to flexion deformity at knee and ankle. His wrist and right ankle knee arthritis improved over time with treatment. There were subcutaneous nodules over left leg and both arms. He also noted left elbow weakness and decreased sensations in upper left arm, 2 months after the onset of first episode of acute pancreatitis. Examination at recent admission revealed normal vital parameters, hyperextended left elbow with poorly demarcated sensory loss in left arm, left knee and bilateral ankle joint effusion with active synovitis with decreased range of motion, however small joints and spine were normal. There were multiple 0. 5- to 1-cm sized nodular lesions in both forearms and left leg. Abdominal examination was grossly normal except moderate tenderness at epigastrium with no rebound tenderness. Biopsy of skin nodule showed panniculitis. Laboratory examination was negative for igG4, autoimmune disorders. Imaging confirmed the diagnosis of acute recurrent pancreatitis with polyarticular involvement. Systemic (methylprednisolone, 60 mg daily) and local (intrarticular) steroid therapy resulted in a modest improvement of pain but no improvement in joint deformity. For Plexopathy conservative treatment was given in consultation with neurologist with short course of steroids, pregabalin and amitriptyline but without significant improvement.

PM0097: Rare manifestation of a rare disease. (Anti PL12 presenting as acute ILD with fulminant inflammatory myopathy)

Shaloo Bhasin, Prasan Deep Rath, Swetal Pandey, Rahul Bisaralli; Max Superspecialty Hospital Saket, New Delhi, India

Case Report: 69 years female with seropositive inflammatory poly arthritis presented withchief complains of diffuse myalgias for1 month and acute onsetdyspnea for 1 day. Initial investigations revealed leucocytosis, transaminitis with normal renal, procalcitonin levels. CPK levels were very high. (16340) with a normal urineexamination. Xray chest was s/o extensive b/l fluffy shadows s/o ARDS.

In view of severe respiratory failure patient wasintubated and ventilated and started on iv antibiotics. Bronchoscopy was done, however patient's condition deteriorated further withhigh Fio2inotropic support. During hospital stay patient developed acute ischemia of right upper limb and lower limb with gangrene. Anticoagulation was started for the same.

Further investigations revealed a rapidly rising cpk levels (upto 89 000). RF / Anticcp were high(119 /113). ANA by IF and LIA was negative. Myositis LIA revealed PL12 +++. APLA workup was negative. All cultures including BAL were negative. In view of persistent rising CPK and no clinical improvement a possible diagnosis of acute necrotizinginflammatory myopathy was consideredand patient was started on IVIG. Meanwhile a muscle biopsy was performed which was consistent with inflammatory myopathy. Patient was treated with solumedrol pulse 500mg for 3 days. Her clinical condition gradually improved with clearing of the xray infiltrates and settling of CPK levels.

Discussion: Antisynthetase syndrome (ASS)is characterized by inflammatory myositis associated with ILD. Anti JO-1 is the most common antisynthetase autoantibody, other antisynthtaseantibody namely anti-alanyl-t–RNA synthetase(anti PL 12) has been described. Unlike anti-jo 1 ASS, anti PL12 ASS has a constant lung involvement and gastrointestinal complicationswhereas muscular involvement is less frequent with less severity. Other symptoms includingraynauds and mechanic hands are also rare.

Conclusion: Anti-alanyl-t–RNA synthetase(anti PL 12) are particularly rare antisynthetase antibodies. And should always be considered in patients presenting with Acute ILDand fulminant myositis with no other physical features of typical ASS.

PM0098: Hypokalemic paralysis secondary to Sjogren's interstitial nephritis: A missed opportunity

P Aparna; AIIMS, Bhopal, Madhya Pradesh, India

Introduction: Sjogren's syndrome is an autoimmune disease with glandular and extra glandular manifestations. Tubulointerstitial nephritis is the main renal involvement associated with primary Sjogren syndrome. It can manifest as distal renal tubular acidosis (RTA) which can cause hypokalemia and patient can present with recurrent, fluctuating but reversible episodes of myopathic weakness. RTA has been reported in 4. 3 to 9% of pSS patients; it is more common in middle-aged women, and two-thirds of them will develop symptoms. RTA with hypokalemic paralysis as a presenting feature of Primary Sjogren's syndrome is described in few case reports in literature. It can be the initial presentation in seven percent of Sjogren's patients. Here we describe 2 case reports in support of these.

Case Reports: A 19/F presented to the medicine department and a 34/M presented to neurology department with h/o of progressive weakness of bilateral upper and lower extremities. Neurological examination revealed severe flaccid weakness. Both patients had past medical history was positive for repeated hospital admissions following episodes of weakness and fatigue associated with hypokalemia that responded well to supplemental potassium alone.

Laboratory Results: demonstrated severe hypokalemia with hyperchloremic metabolic acidosis and raised urinary PH. USG abdomen was suggestive of bilateral nephrocalcinosis. EMG/ NCV was done and ruled out AIDP. Immunologic work-up showed a strongly positive ANA and positiveantibodies to SSA and SSB. Schirmer's test was abnormal in one patient, normal in other. Lip biopsy was done and the features were consistent with lymphoplasmacytic sialadenitis compatible with the clinical diagnosis of Sjogren's syndrome. Both patients were treated with steroids and potassium and bicarbonate supplementation, steroids were gradually tapered and stopped and syrup potchlor and bicarbonate were continued. On further follow up, there was gradual improvement in serum potassium with normalization of serum pH and bicarbonate levels and both patients became asymptomatic on these treatment line.

Discussion: The mechanism of hypokalemia in Sjogren's syndrome is because of distal RTA brought about by chronic interstitial nephritis which in turn leads to decrease tubular sodium delivery, defective H-K ATPase, secondary hyperaldosteronism, and bicarbonaturia.

If correctly diagnosed, it is easily treated with immunomodulators, with potassium supplementation and acidosis control with bicarbonates Madhya Pradesh

Conclusion: There is a usual delay in the diagnosis of sjogrens in such presentations as occurred with both patients in our study. This reinforces to keep sjogrens syndrome as DD in recurrent hypokalemicmuscle weakness, especially in the presence of nephrocalcinosis and distal RTA.