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Systemic Sclerosis‐Associated Myopathy
Author(s) -
RANQUE BRIGITTE,
AUTHIER FRANÇOISJÉRÔME,
BEREZNE ALICE,
GUILLEVIN LOÏC,
MOUTHON LUC
Publication year - 2007
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1196/annals.1422.029
Subject(s) - dermatomyositis , medicine , myopathy , polymyositis , pathology , pathological , inflammatory myopathy , microangiopathy , muscle weakness , fibrosis , proximal muscle weakness , pathophysiology , myositis , skeletal muscle , cardiomyopathy , heart failure , muscle biopsy , endocrinology , diabetes mellitus , biopsy
: Skeletal muscle involvement is a common feature in systemic sclerosis (SSc) because muscle weakness is found in up to 90% SSc patients when systematically assessed. Muscle clinical, biological, and electromyographic features are similar to those of polymyositis or dermatomyositis, except for a higher proportion of mild symptoms. SSc‐associated myopathy is more prevalent in diffuse SSc and is also associated with cardiomyopathy. The pathophysiological process leading to SSc‐associated myopathy is likely to be complex, given the heterogeneity of pathological muscle findings, including stigma of microangiopathy, and also inflammatory infiltrate in about half of the cases and interstitial fibrosis. Conflicting results have been reported regarding the correlation between clinicobiological presentation and pathological muscle features, nevertheless there is a general agreement that histologically proven inflammatory myopathies usually regress under high‐dose corticosteroid therapy, or even low dose in case of positive anti‐PM/Scl antibody. In contrast, noninflammatory myopathies often result in milder clinical expression but do not respond to immunosuppressive treatment.