PT  - JOURNAL ARTICLE
AU  - B Ranque
AU  - F-J Authier
AU  - V Le-Guern
AU  - C Pagnoux
AU  - A Berezne
AU  - Y Allanore
AU  - D Launay
AU  - E Hachulla
AU  - A Kahan
AU  - J Cabane
AU  - R Gherardi
AU  - L Guillevin
AU  - L Mouthon
TI  - A descriptive and prognostic study of systemic sclerosis-associated myopathies
AID  - 10.1136/ard.2008.095919
DP  - 2009 Sep 01
TA  - Annals of the Rheumatic Diseases
PG  - 1474--1477
VI  - 68
IP  - 9
4099  - http://ard.bmj.com/content/68/9/1474.short
4100  - http://ard.bmj.com/content/68/9/1474.full
SO  - Ann Rheum Dis2009 Sep 01; 68
AB  - 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.