Display Settings:

Format

Send to:

Choose Destination
Best Pract Res Clin Rheumatol. 2009 Oct;23(5):665-78. doi: 10.1016/j.berh.2009.07.007.

Juvenile dermatomyositis: new developments in pathogenesis, assessment and treatment.

Author information

  • 1Rheumatology Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK. l.wedderburn@ich.ucl.ac.uk

Abstract

Juvenile dermatomyositis (JDM) is a rare, potentially life-threatening systemic autoimmune disease primarily affecting muscle and skin. Recent advances in the recognition, standardised assessment and treatment of JDM have been greatly facilitated by large collaborative research networks. Through these networks, a number of immunogenetic risk factors have now been defined, as well as a number of potential pathways identified in the aetio-pathogenesis of JDM. Myositis-associated and myositis-specific autoantibodies are helping to sub-phenotype JDM, defined by clinical features, outcomes and immunogenetic risk factors. Partially validated tools to assess disease activity and damage have assisted in standardising outcomes. Aggressive treatment approaches, including multiple initial therapies, as well as new drugs and biological therapies for refractory disease, offer promise of improved outcomes and less corticosteroid-related toxicity.

PMID:
19853831
[PubMed - indexed for MEDLINE]
PMCID:
PMC2774891
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for Elsevier Science Icon for PubMed Central
    Loading ...
    Write to the Help Desk