皮肌炎相关间质性肺病发病机制研究进展
Recent Advances in the Pathogenesis of Dermatomyositis-Associated Interstitial Lung Disease
DOI: 10.12677/acm.2026.1631188, PDF,   
作者: 卯 奕, 李晓岚：昆明医科大学第二附属医院皮肤病与性病科，云南 昆明
关键词: 皮肌炎；间质性肺病；自身免疫；干扰素I型；Dermatomyositis； Interstitial Lung Diseases； Autoimmunity； Interferon Type I
摘要: 皮肌炎(dermatomyositis, DM)是一种以皮肤损害和肌无力为典型表现的系统性自身免疫性疾病，其并发症间质性肺病(dermatomyositis-associated interstitial lung disease, DM-ILD)显著影响患者的生活质量并决定远期预后。近年来，对DM-ILD的研究不断深入，但其发病机制尚未完全阐明。现有证据提示，DM-ILD的发生发展与异常自身免疫应答、I型干扰素(type I interferon, IFN-I)通路持续激活以及肺组织损伤后修复失衡密切相关。本文旨在系统梳理上述机制，以期为DM-ILD的早期识别、风险评估及潜在治疗靶点的探索提供理论依据。
Abstract: Dermatomyositis (DM) is a systemic autoimmune disease classically characterized by cutaneous manifestations and muscle weakness. Among its complications, dermatomyositis-associated interstitial lung disease (DM-ILD) markedly compromises patients’ quality of life and is a major determinant of long-term prognosis. In recent years, research on DM-ILD has advanced substantially; however, its pathogenesis has not yet been fully elucidated. Accumulating evidence suggests that the onset and progression of DM-ILD are closely associated with dysregulated autoimmune responses, persistent activation of the type I interferon (IFN-I) signaling pathway, and an imbalance between injury and repair following lung tissue damage. This article aims to systematically synthesize these mechanisms, with the goal of providing a theoretical basis for early identification, risk stratification, and the exploration of potential therapeutic targets in DM-ILD.
文章引用：卯奕, 李晓岚. 皮肌炎相关间质性肺病发病机制研究进展[J]. 临床医学进展, 2026, 16(3): 3799-3808. https://doi.org/10.12677/acm.2026.1631188

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