a Division of Pediatric Rheumatology , Dalhousie University , Halifax , Nova Scotia , Canada.
Expert Rev Clin Immunol. 2018 Dec;14(12):1021-1028. doi: 10.1080/1744666X.2018.1535901. Epub 2018 Oct 22.
Juvenile dermatomyositis (JDM) is a rare, chronic autoimmune illness with primary features of symmetric, proximal muscle weakness and involvement of the skin with a number of identifiable rashes. Evidence to support treatment decisions is limited, given the paucity of clinical trials. Consensus based methods, informed by available data, play an important role in treatment recommendations. Areas covered: This review focuses on evidence and consensus opinion regarding therapeutic options in JDM and identifies gaps where future research is needed. Expert commentary: The combination of trial evidence (as limited as it is) and consensus opinion support standard initial management for children with JDM to consist of high-dose corticosteroids, either intravenous or oral, and methotrexate. Several other agents have preliminary support, either through clinical trials or case series for their use in patients who either fail to respond adequately, have severe disease or have contraindications to standard initial therapy. One of the important goals of management in JDM will be to reduce the corticosteroid exposure experienced by patients. To meet this goal, progress in a number of key areas is needed: increased international collaboration, advances in study design and increased translational research.
幼年特发性关节炎（JDM）是一种罕见的慢性自身免疫性疾病，主要表现为对称性、近端肌无力，伴有多种可识别的皮疹。由于临床试验较少，因此支持治疗决策的证据有限。基于现有数据的共识方法在治疗建议中起着重要作用。
本综述重点关注 JDM 治疗选择的证据和共识意见，并确定了需要进一步研究的空白领域。
试验证据（尽管有限）和共识意见的结合支持对 JDM 患儿进行标准初始治疗，包括高剂量皮质类固醇，无论是静脉注射还是口服，以及甲氨蝶呤。其他几种药物也有初步的支持，无论是通过临床试验还是病例系列，都可用于对标准初始治疗反应不佳、疾病严重或有禁忌症的患者。JDM 管理的一个重要目标将是减少患者的皮质类固醇暴露。为了实现这一目标，需要在多个关键领域取得进展：增加国际合作、改进研究设计和增加转化研究。
