Rheumatology Unit, Institute of Child Health, University College London, 30 Guilford Street, London WC1N 1EH, UK.
Best Pract Res Clin Rheumatol. 2009 Oct;23(5):665-78. doi: 10.1016/j.berh.2009.07.007.
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.
幼年特发性关节炎（JDM）是一种罕见的、潜在危及生命的系统性自身免疫性疾病，主要影响肌肉和皮肤。通过大型合作研究网络，JDM 的识别、标准化评估和治疗方面取得了重大进展。通过这些网络，现在已经确定了一些免疫遗传风险因素，以及 JDM 的发病机制中的一些潜在途径。肌炎相关和肌炎特异性自身抗体有助于根据临床特征、结局和免疫遗传风险因素对 JDM 进行亚表型分类。部分验证的评估疾病活动度和损伤的工具有助于标准化结局。包括多种初始治疗在内的积极治疗方法，以及用于难治性疾病的新型药物和生物疗法，有望改善结局并减少与皮质类固醇相关的毒性。
