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Review
. 2021 Nov;47(4):669-690.
doi: 10.1016/j.rdc.2021.07.003. Epub 2021 Sep 2.

Updates on Juvenile Dermatomyositis from the Last Decade: Classification to Outcomes

Hanna Kim  1 , Adam M Huber  2 , Susan Kim  3
Affiliations
Review

Updates on Juvenile Dermatomyositis from the Last Decade: Classification to Outcomes

Hanna Kim et al. Rheum Dis Clin North Am. 2021 Nov.

Abstract

Juvenile dermatomyositis (JDM) is a heterogeneous disease with new classification criteria and updates in myositis-specific autoantibody and myositis-associated antibody groups. There are many validated assessment tools for assessing disease activity in JDM. Future studies will optimize these tools and improve feasibility in clinical and research contexts. Genetic and environmental risk factors, mechanisms of muscle pathology, role of interferon, vascular markers, and changes in immune cells provide insights to JDM pathogenesis. Outcomes have improved, but chronic disease, damage, and mortality highlight the need for better outcome predictors and treatments. Increased collaboration of stakeholders may help overcome research barriers and improve JDM treatment.

Keywords: Biologic therapies; Juvenile dermatomyositis (JDM); Juvenile myositis (JM); Myositis-specific autoantibodies (MSA); Outcomes; Pathogenesis; Treatment.

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Figures

Fig. 1.
Fig. 1.
Recommended consensus treatment plans from CARRA, for JDM patients with moderate, skin-predominant, and skin-resistant disease. IV: intravenous, IG: immunoglobulin. a: Lesser of 15 mg/m2 or 1 mg/kg (max 40 mg), b: max 60mg, c: optional, d: max 70 grams, e: max 400mg, f: max 1500mg bid, g: higher doses based on toxicity, efficacy
Fig. 2.
Fig. 2.
Recommendations from SHARE, for treatment of JDM, ranging from mild to severe disease. * such as major organ involvement / extensive ulcerative skin disease; ** improvement based on clinical opinion, DMARD: disease modifying antirheumatic drug, MMF: mycophenolate mofetil, MTX: methotrexate
See this image and copyright information in PMC

References

    1. Rider LG, Shah M, Mamyrova G, et al. The myositis autoantibody phenotypes of the juvenile idiopathic inflammatory myopathies. Medicine (Baltimore) 2013; 92(4):223–43. - PMC - PubMed
    1. Tansley SL, Simou S, Shaddick G, et al. Autoantibodies in juvenile-onset myositis: their diagnostic value and associated clinical phenotype in a large UK cohort. J Autoimmun 2017;84:55–64. - PMC - PubMed
    1. Rider LG, Katz JD, Jones OY. Developments in the classification and treatment of the juvenile idiopathic inflammatory myopathies. Rheum Dis Clin North Am 2013;39(4):877–904. - PMC - PubMed
    1. Bohan A, Peter JB. Polymyositis and dermatomyositis (second of two parts). N Engl J Med 1975;292(8):403–7. - PubMed
    1. Lundberg IE, Tjarnlund A, Bottai M, et al. 2017 European League Against Rheumatism/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups. Ann Rheum Dis 2017;76(12):1955–64. - PMC - PubMed

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