The clinicoserological spectrum of inflammatory myopathy in the context of systemic sclerosis and systemic lupus erythematosus

Abstract

Autoimmune rheumatic diseases (ARDs) are characterized by a pathological triad composed of autoimmunity/inflammation, microangiopathy, and aberrant tissue remodeling. Disease terms such as idiopathic inflammatory myopathy (IIM), scleroderma/systemic sclerosis (SSc), and systemic lupus erythematosus (SLE) are helpful clinically but disguise the considerable overlap that exists within these “distinct” disorders. This is perhaps best demonstrated by inflammatory myopathy, which can be present in SSc or SLE, but can itself be absent in clinically amyopathic IIM. Archetypal clinical manifestations of ARD (such as Raynaud's phenomenon) are frequently present, albeit with varying prominence, within each of these diseases. This is certainly the case for inflammatory myositis, which has long been recognized as an important clinical feature of both SSc and SLE. Progress in elucidating the clinicoserological spectrum of ARDs has identified autoantibody specificities that are strongly associated with “overlap” disease and the presence of inflammatory myositis in SSc and SLE. In this review, we shall describe the prevalence, burden, prognostic value, and management considerations of IIM in the context of both SSc and SLE. A major emphasis on the value of autoantibodies shall highlight the value of these tools in predicting the future occurrence of inflammatory myositis in both SSc and SLE. Where applicable, unmet research needs shall be highlighted. The review emphasizes the importance of myopathy as a common feature across all the ARDs and highlights specific antibody specificities that are strongly associated with myopathy in the context of SLE and SSc.

Keywords

• classification
• clinical phenotype
• dermatomyositis
• overlap syndromes
• polymyositis
• systemic lupus erythematosus
• systemic sclerosis