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An update on autoantibodies in the idiopathic inflammatory myopathies

Nature Reviews Rheumatology volume 21, pages 46–62 (2025)Cite this article

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Abstract

Myositis-specific autoantibodies (MSAs) have become pivotal biomarkers for idiopathic inflammatory myopathies and have revolutionized understanding of the heterogeneous disease spectrum that affects both adults and children. The discovery and characterization of MSAs have substantially enhanced patient stratification based on clinical phenotype, thereby facilitating more precise diagnosis and ultimately improving management strategies. Advances in immunoassay technologies in the past 20 years have further propelled the field forward, enabling the detection of a growing repertoire of autoantibodies with high specificity and sensitivity; however, evolving research over the past decade has revealed that even within antibody-defined subsets, considerable clinical diversity exists, suggesting a broader spectrum of disease manifestations than previously acknowledged. Challenges persist, particularly among patients who are seronegative, where the failure to identify certain rare MSAs stems from the use of diverse detection methodologies and inadequate consensus-guided standardization and validation protocols. Bridging these diagnostic gaps is crucial for optimizing patient care and refining prognostic stratification in idiopathic inflammatory myopathies.

Key points

  • Autoantibodies, found in over half of adult and paediatric patients with idiopathic inflammatory myopathies (IIMs), correlate with specific clinical phenotypes, aiding in the classification, diagnosis and prognostic assessment of these diseases.

  • The emergence of new myositis autoantibodies, along with a deeper understanding of serological categories in the past 10 years, enriches the diagnostic and prognostic repertoire for IIMs.

  • A more concerted global exploration of ethnic, environmental and genetic diversities is crucial to improve the characterization of subsets within the serological categories already known in IIMs.

  • Numerous new immunoassays for detecting myositis-specific autoantibodies, including line blot techniques, have variable performance and lack standardized protocols, requiring further validation for reliable myositis autoantibody detection.

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Fig. 1: Autoantibodies associated with different IIM manifestations.

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Acknowledgements

L.R.W. is supported by the National Institute for Health Research (NIHR) via the NIHR-Biomedical Research Centre at Great Ormond Street Hospital and an NIHR Senior Investigator award. The views expressed are those of the author and not necessarily those of the National Health Service, the NIHR or the Department of Health and Social Care.

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Authors and Affiliations

  1. Rheumatology Service, Department of Medicine, Woodlands Health, Singapore, Singapore

    Nur Azizah Allameen

  2. Department of Rheumatology, Hospital General Universitario Gregorio Marañón, Madrid, Spain

    Ana Isabel Ramos-Lisbona

  3. Inflammation and Rheumatology Section, UCL Great Ormond Street Institute of Child Health, London, UK

    Lucy R. Wedderburn

  4. UK Centre for Adolescent Rheumatology Versus Arthritis at UCL, University College Hospital and Great Ormond Street Children’s Hospital, London, UK

    Lucy R. Wedderburn

  5. Department of Rheumatology, Great Ormond Street Children’s Hospital, London, UK

    Lucy R. Wedderburn

  6. Division of Rheumatology, Department of Medicine, Solna, Karolinska Institutet, Stockholm, Sweden

    Ingrid E. Lundberg

  7. Department of Gastroenterology, Dermatology and Rheumatology, Theme Inflammation and Aging, Karolinska University Hospital, Stockholm, Sweden

    Ingrid E. Lundberg

  8. Department of Ageing, Rheumatology and Regenerative Medicine, Division of Medicine, University College London, London, UK

    David A. Isenberg

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  1. Nur Azizah Allameen
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D.A.I., N.A.A. and A.I.R.-L. researched data for the article and contributed substantially to discussion of the content. All authors wrote the article and reviewed and/or edited the manuscript before submission.

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Allameen, N.A., Ramos-Lisbona, A.I., Wedderburn, L.R. et al. An update on autoantibodies in the idiopathic inflammatory myopathies. Nat Rev Rheumatol 21, 46–62 (2025). https://doi.org/10.1038/s41584-024-01188-4

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