Article Text

Download PDFPDF
Autoantibody testing in idiopathic inflammatory myopathies
  1. Anke Rietveld1,
  2. Johan Lim2,
  3. Marianne de Visser2,
  4. Baziel van Engelen1,
  5. Ger Pruijn3,
  6. Olivier Benveniste4,
  7. Anneke van der Kooi2,
  8. Christiaan Saris1
  1. 1 Department of Neurology, Center for Neuroscience Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands
  2. 2 Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, Amsterdam, The Netherlands
  3. 3 Department of Biomolecular Chemistry, Radboud Institute for Molecular Life Sciences and Institute for Molecules and Materials, Nijmegen, The Netherlands
  4. 4 Médecine Interne et Immunologie Clinique, Centre de Référence Maladies Neuro-Musculaires, Sorbonne Université, AP-HP, GH Pitié-Salpêtrière, UMR974, Paris, France
  1. Correspondence to Dr Anke Rietveld, Department of Neurology, Center for Neuroscience Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen 6500 HB, The Netherlands; Anke.Rietveld{at}


The diagnosis and classification of idiopathic inflammatory myopathies are based mainly on clinical and histological features. The discovery of myositis-specific and myositis-associated antibodies has simplified the (sub)classification of inflammatory myopathies. Patients suspected of having an idiopathic inflammatory myopathy should undergo routine antibody testing to gain more insight into distinct phenotypes, comorbidities, treatment response and prognosis. Furthermore, autoantibody testing can help in patients with atypical patterns of weakness or with an unresolved limb-girdle myopathic phenotype, or interstitial lung disease. However, some important technical and methodological issues can hamper the interpretation of antibody testing; for example, some antibodies are not included in the widely available line blots. We aim to provide a practical review of the use of autoantibody testing in idiopathic inflammatory myopathies in clinical practice.

  • incl body myositis
  • polymyositis
  • neuroimmunology
  • neuromuscular

Statistics from

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.


  • Contributors AR and JL wrote the initial version of the manuscript, which was reviewed and partially rewritten by MdV, AvdK, BvE, OB, GP and CS.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests GP and BvE are inventors of a patent (EP20120740236) licensed to Euroimmun AG, and GP received financial support from Euroimmun for his research programme. BvE reports a grant from Prinses Beatrix Spierfonds and personal fees and non-financial support from Fulcrum, personal fees from Facio, and grants from European Union’s Horizon 2020 research and innovation programme (Murab), Netherlands Organisation for Scientific Research (NWO), The Netherlands Organisation for Health Research and Development (ZonMw), Global FSH, Stichting Spieren voor Spieren, Dutch FSHD Foundation, and Association Française contre les Myopathies, outside the submitted work. JL and AvdK report grants from CSL Behring for an interventional study, outside the scope of this review. MdV serves as a consultant for Bristol-Myers Squibb. OB serves as a consultant for Novartis, Neovacs and CSL Behring.

  • Patient consent for publication Obtained.

  • Provenance and peer review Commissioned; externally peer reviewed by Jon Walters, Swansea, UK.

Linked Articles

  • Editors’ commentary
    Phil E M Smith Geraint N Fuller

Other content recommended for you