Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients
Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients
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DOI：
10.1590/s0004-282x2002000600001
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发表时间：
2002-12-01
影响因子：
1.4
通讯作者：
Marie, Suely Kazue Nagahashi
中科院分区：
文献类型：
--
作者：
Sallum, Adriana Maluf Elias;Kiss, Maria Helena Bittencurt;Marie, Suely Kazue Nagahashi
本研究基于对35例符合Bohan和Peter幼年型皮肌炎诊断标准的患者的前瞻性和回顾性分析。平均随访3年10个月。5例（14.28%）患者存在钙质沉着，4例（11.42%）患者存在皮肤溃疡，9例（27.71%）患者存在全身受累。所有患者的血清肌酶水平的变化，他们都提交了肌肉活检作为诊断程序。9例（25.71%）患者在肌肉活检前和26例（74.28%）患者在肌肉活检后接受了皮质激素治疗。对皮质激素治疗效果差的患者采用氯喹、甲氨蝶呤、环孢素、环磷酰胺和静脉注射免疫球蛋白。4例（11.43%）患者的皮肤表现持续存在，1例（2.85%）患者的实验室活动持续存在，3例（8.57%）患者的皮肤和实验室活动持续存在。2002年3月研究终点时，10例（28.57%）患者停止活动，17例（48.57%）患者缓解。死亡2例（5.71%）。
This study was based on a prospective and a retrospective analysis of 35 patients who met Bohan and Peter criteria for juvenile dermatomyositis diagnosis. The mean follow-up time was three years ten months. Calcinosis was present in five (14.28%) patients, cutaneous ulcers in four (11.42%), and systemic involvement in nine (27.71%) patients. All patients presented alterations in the serum levels of muscle enzymes, and all of them were submitted to muscle biopsy as a diagnostic procedure. Nine (25.71 %) patients received corticotherapy prior to and 26 (74.28%) after the muscle biopsy. Chloroquine, methotrexate, cyclosporine, cyclophosphamide and intravenous immunoglobulin were used in patients with poor response to corticotherapy. Continuation of cutaneous manifestations was observed in 4 (11.43%) patients, laboratorial activity in 1 (2.85%), cutaneous and laboratorial activities in 3 (8.57%). Ten (28.57%) patients were out of activity, and 17 (48.57%) in remission at study end-point, on March 2002. Two (5.71%) patients died.