具有自身免疫特征的间质性肺炎的诊断及治疗进展
Progress in the Diagnosis and Treatment of Interstitial Pneumonia with Autoimmune Features
摘要: 具有自身免疫特征的间质性肺炎(Interstitial Pneumonia with Autoimmune Features, IPAF)在研究中被定义为与自身免疫特征相关的间质性肺病(Interstitial lung Disease, ILD)，但未确诊为风湿性疾病。自2015年IPAF标准发布以来，已有多项关于IPAF的研究。目前，关于IPAF的发病机制、预后和治疗仍不清楚。本文详细综述了IPAF的分类，列出了将患者分类为IPAF的挑战，以及IPAF的患病率、流行病学、临床表现、预后，和决定临床病程的重要特征，并综述了其发病机制，并回顾了其治疗策略。
Abstract: Interstitial Pneumonia with Autoimmune Features (IPAF) is defined in research as interstitial lung disease (ILD) with autoimmune features, but not diagnosed as a rheumatic disease. Since the release of the IPAF criteria in 2015, there have been numerous studies on IPAF. Currently, the pathogenesis, prognosis, and treatment of IPAF remain unclear. This article provides a detailed review of the classification of IPAF, outlining the challenges of classifying patients as having IPAF, as well as the prevalence, epidemiology, clinical manifestations, prognosis, and key features that determine the clinical course of IPAF. It also reviews the pathogenesis and treatment strategies.
文章引用：潘登, 陈蒙蒙, 赵连波. 具有自身免疫特征的间质性肺炎的诊断及治疗进展[J]. 临床医学进展, 2026, 16(5): 2814-2820. https://doi.org/10.12677/acm.2026.1652093

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