LAUSR

INTRODUCTION Idiopathic inflammatory myopathies (IIMs) represent a diverse group of systemic autoimmune disorders with variable clinical manifestations and disease course. Currently, the challenges of IIMs are multifold, including difficulties in timely diagnosis owing to clinical heterogeneity, limited insights into disease pathogenesis, as well as a restricted number of available therapies. However, advances utilizing myositis-specific autoantibodies (MSAs) have facilitated the definition of subgroups as well as prediction of clinical phenotypes, disease course, and response to treatment. AREAS COVERED Herein we provide an overview of the clinical presentations of dermatomyositis (DM), anti-synthetase syndrome (ASyS), immune-mediated necrotizing myopathy (IMNM) and inclusion body myositis (IBM). We then provide an updated review of available and promising therapies for each of these disease groups. We synthesize current treatment recommendations in the context of case-based construct to facilitate application to patient care. Finally, we provide high-yield, clinical pearls relevant to each of the subgroups that can be incorporated into clinical reasoning. EXPERT OPINION There are many exciting developments on the horizon for IIM. As insights into pathogenesis evolves, the therapeutic armamentarium is expanding with many novel therapies in development, holding promise for more targeted treatment approaches.