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Updated: Jun 27, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
A descriptive and prognostic study of systemic sclerosis-associated myopathies
B Ranque1, F-J Authier, V Le-Guern
1Université Paris Descartes, UPRES EA 4058, Department of Internal Medicine, French National Reference Center for Systemic Sclerosis and Necrotizing Vasculitides, Hôpital Cochin, Assistance Publique des Hôpitaux de Paris, Paris, France.
Objectives:
To describe the clinical characteristics and muscle pathological features of patients with systemic sclerosis (SSc) and myopathy and analyse their impact on muscle outcome.
Methods:
Thirty-five patients with myopathy and available muscle biopsy were restrospectively investigated from the charts of four hospital centres.
Results:
Twenty-six (74%) cases had diffuse SSc. The median time from SSc diagnosis was 5 years (range 0-23) at myopathy onset. The main myopathological features were mononuclear inflammation (63%), muscle atrophy (60%), necrosis (59%), regeneration (44%), fibrosis (24%) or microangiopathy (27%). After a median follow-up of 4.4 years, 24 patients (69%) showed complete or partial muscle remission. Only histological muscle inflammation was associated with good muscle prognosis in multivariate analysis (odds ratio 44.7, 95% CI 2.8 to 704.7). Patients without muscle inflammation had a poor response to corticosteroids (38% favourable response vs 90% in patients with inflammation).
Conclusion:
Muscle histopathology is critical in the therapeutic management of SSc-associated myopathy.
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