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Epidemiology of the idiopathic inflammatory myopathies

Nature Reviews Rheumatology volume 19, pages 695–712 (2023)Cite this article

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Abstract

The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of systemic autoimmune diseases that affect the skeletal muscles and can also involve the skin, joints, lungs and heart. The epidemiology of IIM is obscured by changing classification criteria and the inherent shortcomings of case identification using healthcare record diagnostic coding. The incidence of IIM is estimated to range from 0.2 to 2 per 100,000 person-years, with prevalence from 2 to 25 per 100,000 people. Although the effects of age and gender on incidence are known, there is only sparse understanding of ethnic differences, particularly in indigenous populations. The incidence of IIM has reportedly increased in the twenty-first century, but whether this is a genuine increase is not yet known. Understanding of the genetic risk factors for different IIM subtypes has advanced considerably. Infections, medications, malignancy and geography are also commonly identified risk factors. Potentially, the COVID-19 pandemic has altered IIM incidence, although evidence of this occurrence is limited to case reports and small case series. Consideration of the current understanding of the epidemiology of IIM can highlight important areas of interest for future research into these rare diseases.

Key points

  • The idiopathic inflammatory myopathies (IIMs) are a heterogeneous group of rare, systemic autoimmune conditions with an incidence of 0.2–2 per 100,000 person-years, and a prevalence of 2–25 per 100,000 people.

  • Changing classification criteria, biases in patient identification and heterogeneity within the group of IIMs affect the ascertainment of incidence and prevalence.

  • The incidence and prevalence of IIM varies with age and gender, and between geographical regions, but is still unreported in many areas, particularly in South America, Africa and Asia.

  • Further research is needed in non-white cohorts of IIM to better understand phenotypic, serological and genetic characteristics of ethnically diverse groups.

  • Although mortality from IIM has improved with earlier diagnosis and treatment, substantial morbidity remains in association with malignancy, cardiovascular events, lung disease and infections, resulting in considerable healthcare costs.

  • Understanding is growing of the unique genetic risk factors and gene–environment risk interactions in IIM, and of the influence of infections, medications (including statins and immune-checkpoint inhibitors), malignancy and geography.

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Fig. 1: Incidence of rheumatic diseases.
Fig. 2: Changing criteria of the idiopathic inflammatory myopathies.
Fig. 3: Incidence and prevalence of adult dermatomyositis and sporadic inclusion-body myositis.

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Acknowledgements

This research was supported by the NIHR Manchester Biomedical Research Centre (NIHR203308). The views expressed are those of the authors and not necessarily those of the NIHR or the Department of Health and Social Care. T.K. is supported by the Ken Muirden ARA Fellowship as part of the 2023 Arthritis Australia National Research Program, and an Australian Government Postgraduate Research Training Program Scholarship.

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Authors and Affiliations

  1. Division of Musculoskeletal and Dermatological Sciences, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, UK

    Thomas Khoo, James B. Lilleker & Hector Chinoy

  2. School of Medicine, Faculty of Health and Medical Sciences, The University of Adelaide, Adelaide, South Australia, Australia

    Thomas Khoo

  3. Department of Rheumatology, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Manchester Academic Health Science Centre, Salford, UK

    Thomas Khoo & Hector Chinoy

  4. Manchester Centre for Clinical Neuroscience, Manchester Academic Health Science Centre, Salford Royal Hospital, Northern Care Alliance NHS Foundation Trust, Salford, UK

    James B. Lilleker

  5. Department of Rheumatology, Allergy and Immunology, Tan Tock Seng Hospital, Singapore, Singapore

    Bernard Yu-Hor Thong

  6. Department of Medicine, Division of Rheumatology, McGill University, Montreal, Canada

    Valérie Leclair

  7. Epidemiology and Public Health Group, School of Health Sciences, The University of Manchester, Manchester, UK

    Janine A. Lamb

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All authors researched data and contributed substantially to discussion of the content. T.K. wrote the article. All authors contributed to reviewing and/or editing the manuscript before submission.

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Khoo, T., Lilleker, J.B., Thong, B.YH. et al. Epidemiology of the idiopathic inflammatory myopathies. Nat Rev Rheumatol 19, 695–712 (2023). https://doi.org/10.1038/s41584-023-01033-0

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