Myopathy in scleroderma, its identification, prevalence, and treatment: lessons learned from cohort studies

Curr Opin Rheumatol. 2014 Mar;26(2):124-30. doi: 10.1097/BOR.0000000000000024.


Purpose of review: This review discusses the characterization of myopathy in scleroderma with a focus on new developments in imaging, biomarkers, and therapy, and details several current reports and several seminal reports prior to 2012.

Recent findings: In the past year, studies have shown that MRI techniques highlight the importance of muscle edema in scleroderma, and that aldolase may be a useful biomarker to predict incident myopathy. When compared to studies preceding 2012, both the current and prior reports too often fail to account for the full spectrum of muscle disease in scleroderma. There remain no uniform classification criteria that are routinely integrated into clinical research reports. Thus, important questions remain to be answered, including risk factors for developing myopathy, optimal screening and diagnostic strategies, and efficacious therapies. But, just as important is the priority to systematically define what the true entity(ies) of myopathy is in scleroderma.

Summary: Scleroderma myopathy is a heterogeneous group of muscle disorders among patients with underlying scleroderma which requires robust studies to clarify the full spectrum of disease.

Publication types

  • Review

MeSH terms

  • Abatacept
  • Autoantibodies / blood
  • Biomarkers / metabolism
  • Cohort Studies
  • Dermatomyositis / etiology
  • Exoribonucleases / immunology
  • Exosome Multienzyme Ribonuclease Complex / immunology
  • Fructose-Bisphosphate Aldolase / metabolism
  • Humans
  • Immunoconjugates / therapeutic use
  • Magnetic Resonance Imaging
  • Muscular Diseases / diagnosis
  • Muscular Diseases / etiology*
  • Muscular Diseases / therapy
  • Polymyositis / etiology
  • Prevalence
  • Risk Factors
  • Scleroderma, Systemic / complications*
  • Scleroderma, Systemic / immunology
  • Scleroderma, Systemic / therapy


  • Autoantibodies
  • Biomarkers
  • Immunoconjugates
  • Abatacept
  • Exoribonucleases
  • Exosome Multienzyme Ribonuclease Complex
  • EXOSC10 protein, human
  • Fructose-Bisphosphate Aldolase