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A descriptive and prognostic study of systemic sclerosis-associated myopathies
  1. B Ranque1,
  2. F-J Authier2,
  3. V Le-Guern1,
  4. C Pagnoux1,
  5. A Berezne1,
  6. Y Allanore3,
  7. D Launay4,
  8. E Hachulla4,
  9. A Kahan3,
  10. J Cabane5,
  11. R Gherardi2,
  12. L Guillevin1,
  13. L Mouthon1
  1. 1
    Université Paris Descartes, Faculté de Médecine, 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 (AP-HP), Paris, France
  2. 2
    Reference Center for Neuro-muscular Diseases, Hôpital Henri Mondor, AP-HP; INSERM U841, E10; Université Paris 12, Créteil, France
  3. 3
    Université Paris Descartes, Faculté de Médecine, Department of Rheumatology A, Hôpital Cochin, AP-HP, Paris, France
  4. 4
    Department of Internal Medicine, French National Reference Center for Systemic Sclerosis, Hôpital Regional Universitaire Claude-Huriez, Université Lille 2, 59037 Lille Cedex, France
  5. 5
    Université Paris VI, Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Paris, France
  1. Correspondence to Dr L Mouthon, Department of Internal Medicine, Cochin Hospital, 27 rue du Faubourg Saint-Jacques, 75679 Paris Cedex 14, France; luc.mouthon{at}


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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  • ▸ Additional supplemental table is published online only at

  • Competing interests None.

  • Ethics approval Ethics approval was obtained.

  • DL, EH, JC, YA, AB, AK, LG and LM are members of the Groupe Français de Recherche sur la Sclérodermie.

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