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Management Considerations: Juvenile Dermatomyositis

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Managing Myositis

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Abstract

Treatment recommendations for juvenile dermatomyositis (JDM) mostly rely on experience, both personal and shared, as well as that published in the literature, most often case reports and small case series. Despite the lack of any randomized trials, glucocorticoids remain the mainstay of therapy for JDM. However, the side effect profile of long-term glucocorticoids is undesirable, and steroid-sparing immunosuppressive and biologic therapies are necessary. Early aggressive intervention is important in limiting JDM-related morbidity, and collaborative research approaches including the Paediatric Rheumatology International Trials Organization (PRINTO), Childhood Arthritis and Rheumatology Research Alliance (CARRA), and International Myositis Assessment and Clinical Studies Group (IMACS) are essential. These organizations utilize prospectively collected data for use in comparative research. A better understanding of the pathogenesis of JDM is driving the search for improved therapies for JDM. Autologous stem cell transplantation (aSCT) is reserved for those patients with severe refractory disease and has shown promise in re-establishing immune tolerance in the small number of JDM patients treated to date. Exercise therapy is safe and includes aerobic and resistance training.

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  1. Department of Pediatrics, Duke University School of Medicine, Durham, NC, USA

    Jeffrey Dvergsten & Ann Reed

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    Rohit Aggarwal

  2. Division of Rheumatology and Clinical Immunology, University of Pittsburgh, School of Medicine, Pittsburgh, PA, USA

    Chester V. Oddis

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Dvergsten, J., Reed, A. (2020). Management Considerations: Juvenile Dermatomyositis. In: Aggarwal, R., Oddis, C. (eds) Managing Myositis. Springer, Cham. https://doi.org/10.1007/978-3-030-15820-0_30

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