Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood

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

Research output: Contribution to journalArticle

251 Citations (Scopus)

Abstract

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
Volume371
Issue number9631
DOIs
StatePublished - 2008

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Myositis
HLA-B8 Antigen
Immunogenetics
Muscles
Interferon Type I
Immunosuppressive Agents
Exanthema
Methotrexate
Dendritic Cells
Haplotypes
Adrenal Cortex Hormones
Up-Regulation
Randomized Controlled Trials
Parturition
Morbidity
Infection
Genes
Juvenile dermatomyositis

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Feldman, B. M., Rider, L. G., Reed, A. M., & Pachman, L. M. (2008). Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. The Lancet, 371(9631), 2201-2212. https://doi.org/10.1016/S0140-6736(08)60955-1

Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. / Feldman, Brian M.; Rider, Lisa G.; Reed, Ann M.; Pachman, Lauren M.

In: The Lancet, Vol. 371, No. 9631, 2008, p. 2201-2212.

Research output: Contribution to journalArticle

Feldman, BM, Rider, LG, Reed, AM & Pachman, LM 2008, 'Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood', The Lancet, vol. 371, no. 9631, pp. 2201-2212. https://doi.org/10.1016/S0140-6736(08)60955-1
Feldman, Brian M. ; Rider, Lisa G. ; Reed, Ann M. ; Pachman, Lauren M. / Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhood. In: The Lancet. 2008 ; Vol. 371, No. 9631. pp. 2201-2212.
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