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青少年皮肌炎:35例患者的临床、实验室、组织学、治疗及转归参数

Juvenile dermatomyositis: clinical, laboratorial, histological, therapeutical and evolutive parameters of 35 patients.

作者信息

Sallum Adriana Maluf Elias, Kiss Maria Helena Bittencurt, Sachetti Silvana, Resende Maria Bernadate Dutra, Moutinho Kelly Cristina, Carvalho Mary de Souza, Silva Clovis Arthur Almeida, Marie Suely Kazue Nagahashi

机构信息

Pediatric Rheumatology Unait of Pediatric Department, University of São Pablo Medical School, São Pablo, Brazil.

出版信息

Arq Neuropsiquiatr. 2002 Dec;60(4):889-99. doi: 10.1590/s0004-282x2002000600001. Epub 2003 Jan 15.

Abstract

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.

摘要

本研究基于对35例符合博汉和彼得青少年皮肌炎诊断标准患者的前瞻性和回顾性分析。平均随访时间为三年零十个月。5例(14.28%)患者出现钙质沉着,4例(11.42%)出现皮肤溃疡,9例(27.71%)出现全身受累。所有患者血清肌酶水平均有改变,且均接受了肌肉活检作为诊断程序。9例(25.71%)患者在肌肉活检前接受了皮质激素治疗,26例(74.28%)在肌肉活检后接受了治疗。对皮质激素治疗反应不佳的患者使用了氯喹、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%)患者出现皮肤表现持续存在,1例(2.85%)出现实验室指标异常,3例(8.57%)出现皮肤和实验室指标异常。在2002年3月研究终点时,10例(28.57%)患者病情缓解,17例(48.57%)患者病情缓解。2例(5.71%)患者死亡。

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