Plamondon S, Dent P B
Department of Pediatrics, McMaster University, Children's Hospital, Hamilton Health Sciences Corporation, Ontario, Canada.
J Rheumatol. 2000 Aug;27(8):2031-4.
To review the clinical features of juvenile amyopathic dermatomyositis (ADM) to define an appropriate approach to its diagnosis and management.
Based on a review of published adult and pediatric cases, a prevalidated, peer reviewed, 3 page questionnaire was sent to all members of the Pediatric Section of the American College of Rheumatology and American Society for Pediatric Dermatology.
Thirty-nine questionnaires were submitted for analysis. Twelve cases were excluded due to abnormal test results. Only one case met all criteria. Although 26 cases were incompletely investigated or had inadequate followup, they were not excluded, as all completed tests were normal. Two patients with incomplete data developed calcinosis. Of 27 patients not positively excluded, 10 were treated systemically, with 5 achieving remission, while 11/17 untreated recovered spontaneously. At a mean followup of 32.8 months from disease onset none of the 27 patients has developed clinical myopathy.
The classic skin changes of juvenile DM can occur in the absence of clinical muscle involvement. Physicians are not routinely performing electromyography, muscle biopsy, or magnetic resonance imaging in the assessment of these patients. A significant proportion of patients with ADM will remit without systemic therapy. Optimum treatment needs to be determined through controlled trials.
回顾青少年无肌病性皮肌炎（ADM）的临床特征，以确定其诊断和管理的合适方法。
在回顾已发表的成人和儿童病例的基础上，向美国风湿病学会儿科分会和美国儿科皮肤病学会的所有成员发送了一份经过预验证、同行评审的3页问卷。
共提交了39份问卷进行分析。12例因检查结果异常被排除。仅1例符合所有标准。尽管26例调查不完整或随访不足，但由于所有完成的检查均正常，故未被排除。2例数据不完整的患者出现了钙质沉着。在27例未被明确排除的患者中，10例接受了全身治疗，5例病情缓解，而17例未治疗的患者中有11例自发康复。从疾病发作开始平均随访32.8个月，27例患者均未出现临床肌病。
青少年皮肌炎的典型皮肤改变可在无临床肌肉受累的情况下出现。医生在评估这些患者时通常不进行肌电图、肌肉活检或磁共振成像检查。相当一部分ADM患者未经全身治疗即可缓解。最佳治疗方案需要通过对照试验来确定。
