Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care

Charalampia Papadopoulou, Christine Chew, Meredyth G. Ll Wilkinson, Liza McCann, Lucy R. Wedderburn

Research output: Contribution to journalReview articlepeer-review


The childhood-onset or juvenile idiopathic inflammatory myopathies (JIIMs) are a heterogenous group of rare and serious autoimmune diseases of children and young people that predominantly affect the muscles and skin but can also involve other organs, including the lungs, gut, joints, heart and central nervous system. Different myositis-specific autoantibodies have been identified that are associated with different muscle biopsy features, as well as with different clinical characteristics, prognoses and treatment responses. Thus, myositis-specific autoantibodies can be used to subset JIIMs into sub-phenotypes; some of these sub-phenotypes parallel disease seen in adults, whereas others are distinct from adult-onset idiopathic inflammatory myopathies. Although treatments and management have much improved over the past decade, evidence is still lacking for many of the current treatments and few validated prognostic biomarkers are available with which to predict response to treatment, comorbidities (such as calcinosis) or outcome. Emerging data on the pathogenesis of the JIIMs are leading to proposals for new trials and tools for monitoring disease.

Original languageEnglish
Pages (from-to)343-362
Number of pages20
JournalNature Reviews Rheumatology
Issue number6
Early online date15 May 2023
Publication statusPublished - 1 Jun 2023


  • Autoantibodies
  • Autoimmune Diseases
  • Humans
  • Myositis/diagnosis
  • Phenotype
  • Prognosis


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