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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

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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 progres-sive 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.There-fore,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.

DermatomyositisSigns and symptomsanti-MDA5Clinical protocols

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南京医科大学第一附属医院风湿免疫科,南京 210029

皮肌炎 体征和症状 抗黑色素瘤分化相关基因5 临床方案

2024

中国临床保健杂志
卫生部北京医院 安徽省保健委员会

中国临床保健杂志

CSTPCD
影响因子:0.931
ISSN:1672-6790
年,卷(期):2024.27(4)