Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood

Brian M. Feldman*, Lisa G. Rider, Ann M. Reed, Lauren M. Pachman

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

342 Scopus citations


Juvenile dermatomyositis, the most common inflammatory myopathy of childhood, is a rare systemic autoimmune vasculopathy that is characterised by weakness in proximal muscles and pathognomonic skin rashes. The length of time before the initiation of treatment affects presenting symptoms, laboratory measures, and pathophysiology. It also affects disease outcomes, including the development of pathological calcifications, which are associated with increased morbidity. Both genetic and environmental risk factors seem to have a role in the cause of juvenile dermatomyositis; HLA B8-DRB1*0301 ancestral haplotype is a strong immunogenetic risk factor, and antecedent infections and birth seasonality suggest that environmental stimuli might increase risk. Activation of dendritic cells with upregulation of genes induced by type-1 interferon (α) in muscle and peripheral blood seems to be central to disease pathogenesis. Treatment often includes combinations of corticosteroids, methotrexate, and other immunosuppressive agents. Disease outcome, if treatment is initiated early, is generally good. Randomised controlled trials are needed to define the most effective treatments.

Original languageEnglish (US)
Pages (from-to)2201-2212
Number of pages12
JournalThe Lancet
Issue number9631
StatePublished - 2008

ASJC Scopus subject areas

  • General Medicine


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