抗黑色素瘤分化相关基因5抗体阳性的皮肌炎诊疗进展  

Progress in the diagnosis and treatment of anti-MDA5 positive dermatomyositis

作　　者：谈文峰[1] TAN Wenfeng(Department of Rheumatology,the First Affiliated Hospital of Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院风湿免疫科,南京210029

出　　处：《中国临床保健杂志》2024年第4期433-438,共6页Chinese Journal of Clinical Healthcare

摘　　要：抗黑色素瘤分化相关基因5(MDA5)抗体阳性的皮肌炎是皮肌炎的一种亚型,该类患者易出现间质性肺病,特别是快速进展的间质性肺病(RPILD),发生RPILD后,常对激素和免疫抑制剂治疗产生抵抗,尽管积极治疗,死亡率仍高达70%,严重危害患者生命安全。该文阐述了抗MDA5阳性皮肌炎发病机制、临床特征和治疗进展,还重点介绍了抗MDA5阳性皮肌炎RPILD早期识别和预警方法。Anti-melanoma differentiation-associated gene 5(MDA5)antibody-positive dermatomyositis is a subtype of DM characterized by a high propensity for developing interstitial lung disease,particularly rapidly progressive ILD(RPILD).Patients with RPILD often exhibit resistance to corticosteroid and immunosuppressive treatments,resulting in a high mortality rate of 70%despite active intervention,posing a serious threat to patient survival.Therefore,heightened attention to this condition is warranted.This article reviews the pathogenesis,clinical characteristics,and treatment advancements of anti-MDA5 positive dermatomyositis,with a particular emphasis on early identification and warning methods for RPILD in these patients.

关 键 词：皮肌炎 体征和症状 抗黑色素瘤分化相关基因5 临床方案 

分 类 号：R593.26[医药卫生—内科学]