Skeletal Muscle Involvement

Skeletal myopathy in systemic sclerosis is an evolving area of research and understanding. Previously considered to be a bystander in the clinical manifestations of scleroderma, it is now being recognized that it can lead to severe disability and also mortality. Insights into the pathogenesis have also revealed that there is a spectrum of muscle histopathologic features highlighting not only inflammation but also fibrosis of the myofibers. Novel muscle MRI imaging may also be an important biomarker to assess disease activity in patients with skeletal muscle involvement. Treatment should be directed based on clinical presentation and muscle histopathology.