幼年型皮肌炎相关钙质沉着机制及Janus激酶抑制剂治疗进展

Research progress in the pathogenesis of calcinosis in juvenile dermatomyositis and the treatment with Janus kinase inhibitors

幼年型皮肌炎(JDM)是一种儿童较为罕见的风湿免疫系统疾病。钙质沉着是JDM的标志后遗症之一,最新研究发现Janus激酶(JAK)-信号转导和转录激活因子信号转导途径参与调节炎症诱导的活性氧介导的线粒体钙质沉着,但目前关于JAK抑制剂治疗JDM相关钙质沉着的临床证据较少,现就其作用机制、疗效及安全性进行综述,旨在为该药治疗JDM相关钙质沉着提供依据。

Juvenile dermatomyositis(JDM)is a rare rheumatological autoimmune disease in children.Calcinosis is one of the marked sequelae of JDM.Recent studies have found that the Janus kinase(JAK)-signal transducer and activator of transcription signaling pathway plays a key role in regulating reactive oxygen species-mediated mitochondrial calcinosis induced by inflammation.However,there is little clinical evidence about JAK inhibitors in treating JDM related calcinosis.This review focuses on the mechanism,efficacy and safety of JAK inhibitors,providing a basis for their use in treating calcinosis associated with JDM.