Objectives: To describe clinical characteristics and muscle pathological features of patients with systemic sclerosis (SSc) and myopathy, and analyze their impact on muscle outcome.
Methods: Thirty-five patients with myopathy and available muscle biopsy were identified from the charts of four hospital centers and restrospectively investigated for clinical, biological and histological parameters associated with muscular prognosis.
Results: Twenty-six (74%) cases had diffuse SSc. Median time from SSc diagnosis was 5 years (range 0-23) at myopathy onset. Main myopathological features were mononuclear inflammation (63%), muscle atrophy (60%), necrosis (59%), regeneration (44%), fibrosis (24%) and/or microangiopathy (27%). After a median follow-up of 4.4 years (range 1-11 years), 24 patients (69%) showed complete or partial muscle remission. Among all clinical, biological and pathological features, only histological muscle inflammation was associated with good muscle prognosis in multivariate analysis (OR= 44.7 [2.8-704.7]). In particular, patients without muscle inflammation had a poor response to corticosteroids (38% of favourable response versus 90% in patients with inflammation).
Conclusion: Muscle histopathology is critical in the therapeutic management of SSc-associated myopathy, because patients without muscle inflammation are unlikely to get benefit from corticosteroid therapy.
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