中国临床保健杂志2024,Vol.27Issue(4) :433-438.DOI:10.3969/J.issn.1672-6790.2024.04.001

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

谈文峰
中国临床保健杂志2024,Vol.27Issue(4) :433-438.DOI:10.3969/J.issn.1672-6790.2024.04.001

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

谈文峰1
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作者信息

  • 1. 南京医科大学第一附属医院风湿免疫科,南京 210029
  • 折叠

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.

Abstract

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.

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

Key words

Dermatomyositis/Signs and symptoms/anti-MDA5/Clinical protocols

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出版年

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
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