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Systemic sclerosis-associated myopathy.

Brigitte Ranque1, François-Jérôme Authier, Alice Berezne

  • 1Paris 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, France.

Annals of the New York Academy of Sciences
|September 28, 2007
PubMed
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Systemic sclerosis (SSc) frequently causes muscle weakness, mimicking inflammatory myopathies. While inflammatory SSc-associated myopathy often improves with corticosteroids, noninflammatory types show milder symptoms and do not respond to immunosuppression.

Area of Science:

  • Rheumatology
  • Neurology
  • Pathology

Background:

  • Systemic sclerosis (SSc) commonly affects skeletal muscles, with up to 90% of patients experiencing muscle weakness.
  • SSc-associated myopathy shares features with polymyositis and dermatomyositis but typically presents with milder symptoms.
  • This condition is more frequent in diffuse SSc and may be linked to cardiomyopathy.

Purpose of the Study:

  • To investigate the clinical, biological, and pathological characteristics of SSc-associated myopathy.
  • To understand the complex pathophysiology underlying SSc-associated myopathy.
  • To correlate clinical presentation and pathological findings with treatment response.

Main Methods:

  • Systematic clinical assessment of muscle involvement in SSc patients.

Related Experiment Videos

  • Electromyographic evaluation of muscle function.
  • Histopathological analysis of muscle biopsies, noting microangiopathy, inflammatory infiltrates, and fibrosis.

    • Main Results:

    • Muscle weakness is a prevalent feature in SSc, with diverse clinical and electromyographic presentations.
    • Pathological findings in SSc-associated myopathy are heterogeneous, including microangiopathy, inflammation, and fibrosis.
    • Inflammatory myopathies in SSc generally respond to corticosteroid therapy, particularly with positive anti-PM/Scl antibodies, while noninflammatory types do not.

    Conclusions:

    • SSc-associated myopathy is a complex condition with varied muscle involvement and pathological features.
    • Distinguishing between inflammatory and noninflammatory SSc myopathies is crucial for guiding treatment decisions.
    • Corticosteroid therapy is effective for inflammatory SSc myopathy, whereas noninflammatory forms require different management strategies.