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

Papadopoulou, Charalampia; Chew, Christine; Wilkinson, Meredyth G Ll; McCann, Liza; Wedderburn, Lucy R; (2023) Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care. Nature Reviews Rheumatology , 19 pp. 343-362. 10.1038/s41584-023-00967-9. Green open access

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Abstract

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.

Type: Article
Title: Juvenile idiopathic inflammatory myositis: an update on pathophysiology and clinical care
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1038/s41584-023-00967-9
Publisher version: https://doi.org/10.1038/s41584-023-00967-9
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher's terms and conditions.
Keywords: Idiopathic inflammatory myopathies, Paediatric rheumatic diseases
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10171197
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