TY - JOUR
T1 - The clinicoserological spectrum of inflammatory myopathy in the context of systemic sclerosis and systemic lupus erythematosus
AU - Pauling, John D.
AU - Skeoch, Sarah
AU - Paik, Julie J.
N1 - Funding Information:
JJP is supported by NIAMS K23 AR073927.
Publisher Copyright:
© 2020 Indian Journal of Rheumatology.
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2020/12/1
Y1 - 2020/12/1
N2 - 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.
AB - 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.
KW - Classification
KW - clinical phenotype
KW - dermatomyositis
KW - overlap syndromes
KW - polymyositis
KW - systemic lupus erythematosus
KW - systemic sclerosis
UR - http://www.scopus.com/inward/record.url?scp=85099983381&partnerID=8YFLogxK
U2 - 10.4103/injr.injr_136_20
DO - 10.4103/injr.injr_136_20
M3 - Review article
AN - SCOPUS:85099983381
VL - 15
SP - S81-S90
JO - Indian Journal of Rheumatology
JF - Indian Journal of Rheumatology
SN - 0973-3698
IS - 6
ER -