Stringer Elizabeth, Feldman Brian M
Division of Rheumatology, Department of Pediatrics, The Hospital for Sick Children, 555 University Avenue, Toronto, Ontario, Canada.
Curr Opin Rheumatol. 2006 Sep;18(5):503-6. doi: 10.1097/01.bor.0000240362.32089.4c.
Juvenile dermatomyositis is a rare chronic inflammatory disease that primarily affects the muscles and skin. Immunosuppressive therapy has played a very important role in reducing mortality rates and morbidity. The review focuses on the spectrum of medications currently used in the treatment of juvenile dermatomyositis, highlighting new advances and unanswered questions.
Data regarding the treatment of juvenile dermatomyositis come almost entirely from retrospective studies with relatively small numbers of patients. Corticosteroids continue to be the accepted first-line therapy. Evidence that the addition of methotrexate at initiation of treatment allows corticosteroids to be tapered more rapidly with good outcomes exists. High-risk, refractory patients may benefit from intravenous cyclophosphamide. Results in refractory patients treated with rituximab are also encouraging. Topical immunosuppressant agents have been largely disappointing in treating rash. The effect and role of exercise in the treatment and rehabilitation of patients with juvenile dermatomyositis is an interesting new area of research.
Future research in the treatment of juvenile dermatomyositis should focus on improving the understanding of disease course and its predictors such that treatment protocols can be developed to provide the most benefit and least amount of medication toxicity for the individual patient.
幼年皮肌炎是一种罕见的慢性炎症性疾病，主要累及肌肉和皮肤。免疫抑制疗法在降低死亡率和发病率方面发挥了非常重要的作用。本综述聚焦于目前用于治疗幼年皮肌炎的各类药物，突出新进展和未解决的问题。
关于幼年皮肌炎治疗的数据几乎完全来自患者数量相对较少的回顾性研究。糖皮质激素仍然是公认的一线治疗药物。有证据表明，治疗开始时加用甲氨蝶呤可使糖皮质激素更快减量且效果良好。高危、难治性患者可能从静脉注射环磷酰胺中获益。利妥昔单抗治疗难治性患者的结果也令人鼓舞。局部免疫抑制剂在治疗皮疹方面大多令人失望。运动在幼年皮肌炎患者治疗和康复中的作用是一个有趣的新研究领域。
未来幼年皮肌炎治疗的研究应着重于增进对疾病病程及其预测因素的理解，从而制定治疗方案，为个体患者提供最大益处并使药物毒性降至最低。
