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Review
. 2023 Nov 1;35(6):341-348.
doi: 10.1097/BOR.0000000000000966. Epub 2023 Aug 22.

Myopathy in systemic sclerosis

Caoilfhionn M Connolly  1 , Julie J Paik
Affiliations
Review

Myopathy in systemic sclerosis

Caoilfhionn M Connolly et al. Curr Opin Rheumatol. .

Abstract

Purpose of review: Systemic sclerosis associated myopathy (SSc-AM) is a complex, heterogenous disease that is associated with poor outcomes. SSc-AM lacks a clear definition, and continues to be poorly recognized. The purpose of this review is to provide a contemporary overview of the clinical, serological and pathophysiologic findings in SSc-AM to guide optimal recognition and management of this challenging disease manifestation.

Recent findings: There have been several advances in diagnostic techniques to facilitate characterization of SSc-AM, including muscle MRI, in which findings were correlated to distinct histopathologic categories of muscle involvement in SSc, histopathologic findings of prominent fibrosis or inflammation on biopsy, and the identification of novel autoantibodies associated with SSc-AM, which may be associated with distinct clinical phenotypes. In one of the largest studies to date, 17% of a well phenotyped SSc cohort were found to have myopathy, which was an independent risk of death, even after adjusting for potential confounders, further highlighting the importance of timely recognistion and management of SSc-AM.

Summary: There is increasing recognition of the importance of SSc-AM. Novel diagnostic tools provide the opportunity for more detailed insights into pathophysiologic mechanisms, which may facilitate the development of a rigorous consensus definition of SSc-AM.

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Conflict of interest statement

Conflicts of interest:

None

Figures

Figure 1:
Figure 1:
Diagnostic evaluation for suspected Systemic sclerosis associated myopathy (SSc-AM) Legend: *EMG/NCS electromyography/nerve conduction studies; ^Magnetic resonance imaging
Figure 2:
Figure 2:
Etiologies of Systemic sclerosis associated myopathy (SSc-AM). Legend:*DM dermatomyositis, IMNM immune mediated necrotizing myopathy, PM polymyositis ĈK creatinine kinase #EMG/NCS electromyography/nerve conduction studies
Figure 3:
Figure 3:
Histopathological findings of fibrosing myopathy in SSc-AM Legend:Panel A Hematoxylin and eosin staining. Panel B. Gomori Trichome stain
See this image and copyright information in PMC

References

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