幼年皮肌炎病情评估与治疗进展

摘要

Juvenile dermatomyositis( JDM) is an non-purulent chronic autoimmune disease ,which is the most common type of the juvenile idiopathic inflammatory myopathies. The disease mainly affects skin and mus-cle system,and is characterized by typical skin rash and progressive muscle weakness. At present,the incidence rate of JDM is much lower,and the etiology and pathogenesis of it are not clear. According to the clinical fea-tures and results of the laboratory testing,it is easy to diagnose typical JDM,meanwhile it lacks the unified crite-rion to evaluate the disease activity. And disease assessment is not only the basis of therapy,but also one of the important parameters to evaluate prognosis ;only estimating the disease more accurately can we optimize the treatment,improve the therapeutic effect and prognosis. Therefore,This paper is mainly focused on three topics, including modified diagnosis criteria of JDM,and approaches to the assessment of JDM,and current treatments and new developments in therapy.%幼年皮肌炎是一种非化脓性的慢性自身免疫性疾病,也是幼年特发性炎症性肌病中最常见的一种类型.该病以累及皮肤和骨骼肌为主,特征性皮疹和进行性肌力减退为其主要临床症状.目前幼年皮肌炎发病率较低,其病因、发病机制尚不完全清楚.根据临床症状和实验室检查结果分析,典型幼年皮肌炎不难诊断,但有关疾病活动性评估目前尚缺少统一评价标准.病情评估是制定治疗方案的基础,也是判断预后的重要参数;只有在明确病情的基础上,优化治疗方案,才能提高临床疗效,改善预后.因此该文主要阐述儿童皮肌炎改良诊断、儿童皮肌炎病情评估方法和治疗及其进展等问题.