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A descriptive and prognostic study of systemic sclerosis-associated myopathies
  1. Brigitte Ranque (brigitte.ranque{at}egp.aphp.fr)
  1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, AP-HP, France
    1. Francois-Jérome Authier
    1. Paris 12 University, Reference Center for Neuro-muscular Diseases, Henri Mondor, AP-HP, France
      1. Véronique Le-Guern
      1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, AP-HP, France
        1. Christian Pagnoux
        1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, AP-HP, France
          1. Alice Berezne
          1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, AP-HP, France
            1. Yannick Allanore
            1. Paris Descartes University, Faculty of Medicine, Department of Rheumatology A, Cochin, AP-HP, France
              1. David Launay
              1. Lille 2 University, Department of Internal Medicine,Regional University Claude-Huriez Hospital, France
                1. Eric Hachulla
                1. Lille 2 University, Department of Internal Medicine,Regional University Claude-Huriez Hospital, France
                  1. André Kahan
                  1. Paris Descartes University, Faculty of Medicine, Department of Rheumatology A, Cochin, AP-HP, France
                    1. Jean Cabane
                    1. Paris VI University, Department of Internal Medicine, Saint-Antoine Hospital, AP-HP, France
                      1. Romain Gherardi
                      1. Paris 12 University, Reference Center for Neuro-muscular Diseases, Henri Mondor, AP-HP, France
                        1. Loïc Guillevin
                        1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, APHP, France
                          1. Luc Mouthon (luc.mouthon{at}cch.aphp.fr)
                          1. Paris Descartes University, Faculty of Medicine, Department of Internal Medicine, Cochin, AP-HP, France

                            Abstract

                            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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