期刊
THERAPEUTIC ADVANCES IN MUSCULOSKELETAL DISEASE
卷 4, 期 1, 页码 41-50出版社
SAGE PUBLICATIONS LTD
DOI: 10.1177/1759720X11424460
关键词
dermatomyositis; idiopathic inflammatory muscle disease; juvenile; myopathy
类别
资金
- Cathal Hayes Research Trust
- Wellcome Trust UK [085860]
- Action Medical Research UK [SP4252]
- The Henry Smith Charity
- Arthritis Research UK [14518, 18796]
- Raynaud's and Scleroderma Association
- UK Myositis Support Group
- Great Ormond Street Hospital Children's Charity
Juvenile dermatomyositis (JDM) is a rare but complex and potentially life-threatening autoimmune disease of childhood, primarily affecting proximal muscles and skin. Although the cause of JDM remains unknown it is clear that genetic and environmental influences play a role in the aetiology. In contrast to adults with dermatomyositis, children with JDM are more likely to have complications that are thought to indicate a vasculopathic process, such as severe skin disease, with ulceration or calcinosis, gut vasculopathy or central nervous system disease. New treatments are much needed and are becoming available and being tested through international multicentre trials. This review will focus on recent insights into pathogenesis, the assessment of the disease in children and the modern approach to its treatment.
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