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  • Review Article
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Clinical features, pathogenesis and treatment of juvenile and adult dermatomyositis

Nature Reviews Rheumatology volume 7, pages 664–675 (2011)Cite this article

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Abstract

Juvenile and adult dermatomyositis (DM) have multiple commonalities, yet display differing prevalence of features, outcomes and comorbidities. In general, compared with the disease in adults, children with DM have more vasculopathy and a greater likelihood of calcinosis, periungual and gingival telangiectasias, and ulceration, but have a better long-term prognosis with improved survival. Adults with DM are more likely to have myositis-specific antibodies, develop interstitial lung disease, have amyopathic disease, and have a marked association with malignancy and other comorbidities. Both diseases have similar features on muscle biopsy and interferon gene signature, although subtle differences can exist in pathogenesis and pathology, such as more capillary loss and a greater degree of C5b–9 complement deposition in affected muscle of juvenile patients. Initiatives are underway to improve classification, markers of disease activity and ability to predict outcome of juvenile and adult DM. The purpose of this Review is to compare and contrast the unique features between juvenile and adult disease and to outline new initiatives in the field.

Key Points

  • Despite multiple commonalities between the two diseases, differences between adult and juvenile dermatomyositis (DM) do exist

  • Adults with DM are at an increased risk of malignancy and are more likely to develop interstitial lung disease

  • Juvenile DM is associated with increased vasculopathy, but children and adolescents with DM have improved long-term prognosis and survival

  • Consensus-driven treatment suggestions have been developed to understand best treatments for moderate juvenile DM, but the same consensus-driven treatment is lacking in adults

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Figure 1: Genetics and environmental factors predisposing to juvenile and adult DM.
Figure 2: Early pathological changes in juvenile and adult DM.

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Acknowledgements

The authors would like to acknowledge the support of the State of Minnesota Partnership and the National Institute of Arthritis and Musculoskeletal and Skin Diseases for grant support.

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  1. Department of Pediatrics, Case Western Reserve University Hospital, 11100 Euclid Avenue, Cleveland, 44106, OH, USA

    Angela B. Robinson

  2. Department of Pediatrics and Medicine, Mayo Clinic Rochester, 200 First Street SW, Rochester, 55905, MN, USA

    Ann M. Reed

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Robinson, A., Reed, A. Clinical features, pathogenesis and treatment of juvenile and adult dermatomyositis. Nat Rev Rheumatol 7, 664–675 (2011). https://doi.org/10.1038/nrrheum.2011.139

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