IngentaConnect Systemic Sclerosis-Associated Myopathy

Authors: RANQUE, BRIGITTE¹; AUTHIER, FRANÇOIS-JÉRÔME²; BEREZNE, ALICE¹; GUILLEVIN, LOÏC¹; MOUTHON, LUC¹

Source: Annals of the New York Academy of Sciences, Volume 1108, Number 1, June 2007 , pp. 268-282(15)

Abstract:

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.

Keywords: systemic sclerosis; myopathy; myositis; overlap syndrome

Document Type: Research article

DOI: 10.1196/annals.1422.029

Affiliations: 1: Paris Descartes University, Faculty of Medicine Paris Descartes, UPRES EA 4058, Department of Internal Medicine, French National Reference Center for Systemic Sclerosis and Vasculitides, Cochin Hospital, Assistance Publique des Hôpitaux de Paris (AP-HP), Paris, France 2: Reference Center for Neuro-muscular Diseases, Henri Mondor Hospital, AP-HP, INSERM U 841, Paris 12 University, Créteil, France

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