抗mda5抗体相关皮肌炎的诊疗进展  

Progress in diagnosis and treatment of anti-MDA5 antibody-related dermatomyositis

作　　者：赵江峰[1] 叶霜[1] Zhao Jiangfeng;Ye Shuang(Department of Rheumatology,Renji Hospital,Shanghai Jiao Tong University School of Medicine,Shanghai 201112,China)

机构地区：[1]上海交通大学医学院附属仁济医院风湿科,上海201112

出　　处：《中华检验医学杂志》2021年第11期1070-1075,共6页Chinese Journal of Laboratory Medicine

基　　金：上海市重大临床研究专项(SHDC2020CR1015B);上海市卫生健康委员会面上项目(202040291)。

摘　　要：抗MDA5抗体阳性的皮肌炎(MDA5+DM)是一类罕见的自身免疫性疾病。由于MDA5+DM患者易合并快速进行性间质性肺病(rapidly progressive interstitial lung disease,RP-ILD),故病死率极高。除肺部病变外,MDA5+DM患者还表现有皮肤及肌肉症状。生物学标志物主要包括抗MDA5抗体滴度、铁蛋白、KL-6水平和CD4+CXCR4+T细胞百分比等,可用于MDA5+DM-ILD严重程度、活动性评估、疗效监测,以及预后预测。目前针对该疾病的药物治疗以联合免疫抑制为主。Dermatomyositis(DM)with positive anti-melanoma differentiation-associated gene 5(MDA5)antibodies(MDA5+DM)is a kind of occasional and rare autoimmune disease.Due to the fact that MDA5+DM patients are prone to suffer from the rapid progressive interstitial lung disease(RP-ILD),and the mortality rate is extremely high(all-cause mortality at 6 months is almost 50%).In addition to lung disease,patients with MDA5+DM also suffering from the skin and muscle symptoms.The biomarkers represented by the anti-MDA5 antibody titer,ferritin,KL-6 level and CD4+/CXCR4+T cell percentage are considered to relate with MDA5+DM-ILD′s severity,activity evaluation,therapeutic effect monitoring,and prognosis prediction.The current therapeutic strategies for the disease is mainly combined with immunosuppression.This work systemly summarizes the diagnosis and treatment progress of anti-mda5 antibody-related dermatomyositis,which not only contributes to the research work of related disciplines,but also provides reference for clinical diagnosis and treatment.

关 键 词：皮肌炎 抗MDA5抗体 生物学标记 治疗学 

分 类 号：R593.26[医药卫生—内科学]