title: A descriptive and prognostic study of systemic sclerosis-associated myopathies.
abstract: OBJECTIVE
  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).
  
  
  CONCLUSIONS
  Muscle histopathology is critical in the therapeutic management of SSc-associated myopathy.
authors.affiliation: Université 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.
authors.forename: B
authors.initials: B
authors.lastname: Ranque
authors.forename: F-J
authors.initials: FJ
authors.lastname: Authier
authors.forename: V
authors.initials: V
authors.lastname: Le-Guern
authors.forename: C
authors.initials: C
authors.lastname: Pagnoux
authors.forename: A
authors.initials: A
authors.lastname: Berezne
authors.forename: Y
authors.initials: Y
authors.lastname: Allanore
authors.forename: D
authors.initials: D
authors.lastname: Launay
authors.forename: E
authors.initials: E
authors.lastname: Hachulla
authors.forename: A
authors.initials: A
authors.lastname: Kahan
authors.forename: J
authors.initials: J
authors.lastname: Cabane
authors.forename: R
authors.initials: R
authors.lastname: Gherardi
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.forename: L
authors.initials: L
authors.lastname: Mouthon
mesh_terms: D000328:Adult; D001706:Biopsy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology; D009135:Muscular Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D011379:Prognosis; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications*
chemical_list: D005938:Glucocorticoids