Rheumatology Reports

Pneumocystis jirovecii Pneumonia and Other Infections in Idiopathic Inflammatory Myositis Abstract Purpose of Review The management of patients with idiopathic inflammatory myositis (IIM) can be complex and challenging due to the myriad of complications they can experience. The continued use of corticosteroids, in addition to the rise of combination immunosuppressive therapy, has contributed to the ongoing concern for infection. Perhaps the most feared infection in IIM patients is Pneumocystis jirovecii pneumonia (PJP) given its infrequent occurrence yet high mortality. The field has been, and continues to be, without evidence-based guidelines to help clinicians determine which patients with IIM to prescribe prophylaxis. Herein, we review this literature to provide the clinician with an up-to-date view of infections in IIM. Recent Findings In the past 5 years, a number of studies have been reported highlighting various infectious complications, which help us better understand their frequency and associated risk factors. In addition, data has been published on the potential harms of PJP prophylaxis, to better inform the risk/benefit of our decision-making. Summary Infection remains a major contributor to morbidity and mortality in IIM. A better understanding of which patient subgroups are at risk for particular infections will inform optimal management strategies.

A Comprehensive Update on Kawasaki Disease Vasculitis and Myocarditis Abstract Purpose of the Review Kawasaki disease (KD) is a childhood systemic vasculitis of unknown etiology that causes coronary artery aneurysms (CAA), and if left undiagnosed can result in long-term cardiovascular complications and adult cardiac disease. Up to 20% of KD children fail to respond to IVIG, the mainstay of therapy, highlighting the need for novel therapeutic strategies. Here we review the latest findings in the field regarding specific etiology, genetic associations, and advancements in treatment strategies to prevent coronary aneurysms. Recent Findings Recent discoveries using the Lactobacillus casei cell wall extract (LCWE)-induced KD vasculitis mouse model have accelerated the study of KD pathophysiology and have advanced treatment strategies including clinical trials for IL-1R antagonist, Anakinra. Summary KD remains an elusive pediatric vasculitis syndrome and is the leading cause of acquired heart disease among children in the USA and developed countries. Advancements in combination treatment for refractory KD with further understanding of novel genetic risk factors serve as a solid foundation for future research endeavors in the field.

The Challenges and Opportunities of Classifying Childhood Arthritis Abstract Purpose of Review Childhood arthritis is in need of a new system of classification, owing to deficiencies in the International League of Associations for Rheumatology (ILAR) criteria. We briefly review the history of classification of childhood arthritis, discuss the major criticisms of the current system, and highlight current initiatives to address those concerns. Recent Findings Recent studies in both pediatric and adult rheumatology into the biologic basis of disease as well as the clinical patterns of presentation have informed the efforts toward developing a new classification system. Summary Several efforts are currently underway to improve the classification of childhood arthritis, most notably the project of the Pediatric Rheumatology International Trials Organization (PRINTO). This international alliance of pediatric rheumatologists has begun a 4-step process to create new classification criteria for childhood arthritis. They are currently on step 3 of the process.

Eosinophilic Vasculitis Abstract Purpose of Review Eosinophilic granulomatosis with polyangiitis (EGPA) represents a rare clinical entity, which is getting increasing attention and relevance in view of our better understanding and newer insights into its pathogenesis. Concomitantly better recognition and understanding of the immune pathophysiologic role of eosinophils provide a solid ground of their role on systemic inflammatory disorders and defense against infectious triggers, especially parasites. This review will focus on describing the physiopathology of eosinophils, as well as providing an in depth description of the natural history, clinical spectrum, and therapy of EGPA. Recent Findings Several studies have aimed at finding useful biomarkers to monitor disease activity, and reported data have shown that eotaxin 3, IL25, IL33, and some eicosanoids to be promising options. Regarding therapeutic advances, recently published studies have revealed the efficacy of mepolizumab during induction and maintenance of EGPA. Recently published data confirmed earlier studies that the use of azathioprine during the induction phase is of no benefit during long-term follow-up. In addition, data from the REOVAS study, which uses rituximab, is still ongoing and apparently with promising results. Summary Eosinophils are involved in several systemic inflammatory disorders, and recent gathered data provide support for their role in triggering EGPA. Better understanding of its pathophysiology should generate newer insights into the pathogenesis, biomarkers of disease activity, and therapeutic targets.

Challenges in the Diagnosis and Treatment of Lyme Disease Abstract Purpose of Review Since recognition in 1975, Lyme disease has become the most common vector-borne illness in North America and Europe. The clinical features are well-characterized and treatment is usually curative, but misperceptions about morbidity persist. The purpose of this review is to examine advances in the diagnosis and treatment of Lyme disease, as well as ongoing management challenges. Recent Findings It is useful to recognize that Lyme disease occurs in stages, with early- and late-stage disease. Clinical expression is in part determined by Borrelial variability. For example, some strains of Borrelia burgdorferi, the causative organism in North America, are particularly arthritogenic. Most patients with early Lyme disease can be cured with a single course of oral antibiotic therapy, in contrast to some patients with Lyme arthritis, a late-stage manifestation, who are more antibiotic refractory and require other treatment strategies. Summary Successful treatment of Lyme disease begins with successful diagnosis and with an understanding of the emergence, clinical features, and impact of Lyme disease over the past half century.

Anti-neutrophil Cytoplasmic Antibodies (ANCA) as Disease Activity Biomarkers in a “Personalized Medicine Approach” in ANCA-Associated Vasculitis Abstract Purpose of Review ANCA-associated vasculitides (AAV) are a group of rare diseases characterized by blood vessel inflammation and the presence of circulating anti-neutrophil cytoplasmic antibodies recognizing proteinase-3 (PR3) (PR3-ANCA) or myeloperoxidase (MPO), MPO-ANCA. Recent Findings Historically, ANCAs have been used as biomarkers for disease associations and increases of ANCA levels as predictors of relapse in patients with AAV. Summary In this review, we will summarize and highlight the most recent developments for using ANCA as predictive biomarkers and review some of the important disease-specific features in patients with AAV.

Psoriatic Arthritis: Newer and Older Therapies Abstract Purpose of Review Psoriatic arthritis (PsA) is an immune-mediated systemic inflammatory disorder with heterogeneous clinical features. Treatment for PsA has progressed rapidly, especially over the past two decades. Herein we review relevant studies and key developments in treatment options for PsA from the past 5 years. Recent Findings Conventional disease-modifying anti-rheumatic drugs (DMARDs) such as methotrexate showed some efficacy for several domains of PsA, albeit less than that achieved with TNF inhibitors (TNFi). TNFi have been shown to be efficacious in treatment across all domains of PsA, particularly preventing radiographic damage, and are highly efficient early in the disease course. Inhibitors of IL-12/23, IL-17A, IL-23, phosphodiesterase 4, T cell costimulation, and janus kinases (JAK) have proven efficacious in the treatment of peripheral arthritis of PsA patients. The introduction of biosimilars to TNFi is expected to impact the treatment algorithm for PsA treatment by increasing access to biologic drugs. Newer treatment modalities including IL-23-specific inhibitors, IL-17A and IL-17F dual inhibitors, and jakinibs (janus kinase inhibitors) with different specificity are currently being developed for treatment of PsA. Summary The recent development of new therapeutic agents for PsA has led to better control of PsA across all of its disease domains. The future of PsA management will likely usher in treatment with different mechanisms of action, allow for more access to care, and hopefully see the possibility of precision medicine to help select the optimal treatment approach for individual PsA patients.

Using Registry Data to Understand Disease Evolution in Inflammatory Myositis and Other Rheumatic Diseases Abstract Purpose of Review While rheumatic disease registries collect longitudinal patient information, longitudinal analytic methods are usually not applied to these data. This review will showcase advances in longitudinal designs/analyses, and ways to leverage digital technologies to recruit and retain more registry participants. Recent Findings We will show how the accelerated cohort and longitudinal multiform methods are more efficient than traditional longitudinal designs. We illustrate how a smartphone app is used to recruit participants for a new rheumatic disease registry in the USA. Examples of newer longitudinal techniques applied in myositis and childhood-onset lupus are also presented. Summary Applying high-efficiency longitudinal design and analysis let investigators leverage the rich registry information collected over time. They allow more sophisticated and precise questions to be asked about the disease course of myositis and other rheumatic diseases, which in turn will inform the practice of clinicians and important decisions made by stakeholders.

Application of Non-invasive Imaging in Inflammatory Disease Conditions to Evaluate Subclinical Coronary Artery Disease Abstract Purpose of Review Traditional risk models, such as the Framingham risk score, fail to capture the increased cardiovascular disease risk seen in patients with chronic inflammatory diseases. This review will cover imaging modalities and their emerging applications in assessing subclinical cardiovascular disease for both research and clinical care in patients with chronic inflammatory diseases. Recent Findings Multiple imaging modalities have been studied to assess for subclinical cardiovascular disease via functional/physiologic, inflammatory, and anatomic assessment in patients with chronic inflammatory diseases. Summary The use of imaging to evaluate subclinical cardiovascular disease in patients with chronic inflammatory diseases has the potential to capture early sub-clinical atherosclerosis, to improve risk stratification of future cardiovascular events, and to guide effective disease management.

Juvenile Dermatomyositis—Clinical Phenotypes Abstract Purpose of Review Juvenile dermatomyositis is a heterogeneous disease with variable clinical outcomes. Here, we describe the recognised subtypes of idiopathic inflammatory myositis which occur in children, with particular reference to disease-associated autoantibodies. Recent Findings Large cohort studies have demonstrated that myositis autoantibodies are common in juvenile dermatomyositis and can be found in the majority of patients. They identify homogenous clinical subgroups and inform prognosis, particularly the risks of developing interstitial lung disease. Descriptions of immune-mediated necrotising myositis in juvenile patients have highlighted a rare but important clinical subset typically associated with severe muscle disease and treatment resistance. Summary It is increasingly apparent that autoantibodies can provide detailed information on prognosis and the likely disease associations in those with juvenile dermatomyositis. Further work is needed to establish how this knowledge should influence our approach to treatment.