Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood.

Published

Journal Article (Review)

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 (alpha) 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.

Full Text

Duke Authors

Cited Authors

  • Feldman, BM; Rider, LG; Reed, AM; Pachman, LM

Published Date

  • June 28, 2008

Published In

Volume / Issue

  • 371 / 9631

Start / End Page

  • 2201 - 2212

PubMed ID

  • 18586175

Pubmed Central ID

  • 18586175

Electronic International Standard Serial Number (EISSN)

  • 1474-547X

Digital Object Identifier (DOI)

  • 10.1016/S0140-6736(08)60955-1

Language

  • eng

Conference Location

  • England