| 谈文峰.抗黑色素瘤分化相关基因5抗体阳性的皮肌炎诊疗进展[J].中国临床保健杂志,2024,27(4):433-438. |
| 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 |
| 投稿时间：2024-06-08 |
| DOI：10.3969/J.issn.1672-6790.2024.04.001 |
| 中文关键词: 皮肌炎 体征和症状 抗黑色素瘤分化相关基因5 临床方案 |
| 英文关键词: Dermatomyositis Signs and symptoms anti-MDA5 Clinical protocols 〖FL |
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| 中文摘要: |
| 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。 |
| 英文摘要: |
| 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. |
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