青少年皮肌炎：33例回顾性分析，特别关注肌酸磷酸激酶（CPK）初始水平

Juvenile dermato/polymyositis: a retrospective analysis of 33 cases with special focus on initial CPK levels.

作者信息

Van Rossum M A, Hiemstra I, Prieur A M, Rijkers G T, Kuis W

机构信息

University Hospital for Children and Youth, Het Wilhelmina Kinderziekenhuis, Department of Rheumatology and Immunology, Utrecht, The Netherlands.

出版信息

Clin Exp Rheumatol. 1994 May-Jun;12(3):339-42.

PMID:8070171

Abstract

Retrospective analysis of 33 patients with juvenile dermato/polymyositis showed 45% complete recovery, 26% remission (steroid dependent), 6% wheelchair-dependency, 3% deaths and 10% development of other connective tissue diseases after a mean follow up of 4 years. Patients who received 2 mg/kg of prednisone had the worst prognosis but since they apparently represent a subgroup it is questionable whether high dose prednisone was the cause of the poor prognosis. This subgroup is characterized by high CPK serum levels (a 10-fold increase or more) and an acute type of onset. An initial high ANA titer was found to predict the later development of other connective tissue disorders.

摘要

对33例青少年皮肌炎患者进行回顾性分析，结果显示，平均随访4年后，45%的患者完全康复，26%的患者缓解（依赖类固醇），6%的患者需依靠轮椅行动，3%的患者死亡，10%的患者并发其他结缔组织疾病。接受2mg/kg泼尼松治疗的患者预后最差，但由于他们显然代表一个亚组，高剂量泼尼松是否是预后不良的原因值得怀疑。该亚组的特征是血清肌酸磷酸激酶（CPK）水平较高（升高10倍或更多）且起病急。发现初始抗核抗体（ANA）滴度高可预测其他结缔组织疾病的后期发展。