Lee Cathryn T, Oldham Justin M
Department of Medicine, Section of Pulmonary and Critical Care, University of Chicago, Chicago, IL.
Department of Internal Medicine, Division of Pulmonary, Critical Care, and Sleep Medicine, University of California, Davis, Sacramento, CA.
Clin Pulm Med. 2017 Sep;24(5):191-196. doi: 10.1097/CPM.0000000000000227.
The accurate diagnosis of interstitial lung disease (ILD) is essential for optimal prognostication and management. While connective tissue disease (CTD) is among the most common causes of ILD, some patients have features suggestive of autoimmunity without meeting criteria for a specific CTD. To help define and study this disease entity more uniformly, a 2015 research statement proposed consensus-based criteria and coined the term "interstitial pneumonia with autoimmune features" (IPAF). In this review, we summarize and compare previously proposed criteria to characterize these patients, provide an overview of the IPAF criteria and highlight recent investigations aimed at characterizing IPAF cohorts. We then call attention to questions that have arisen with the application of the IPAF criteria and discuss future areas of study.
间质性肺疾病(ILD)的准确诊断对于优化预后和管理至关重要。虽然结缔组织病(CTD)是ILD最常见的病因之一,但一些患者具有提示自身免疫的特征,却不符合特定CTD的标准。为了更统一地定义和研究这一疾病实体,2015年的一项研究声明提出了基于共识的标准,并创造了“具有自身免疫特征的间质性肺炎”(IPAF)这一术语。在本综述中,我们总结并比较了之前提出的用于描述这些患者的标准,概述了IPAF标准,并强调了旨在描述IPAF队列的最新研究。然后,我们提请注意在应用IPAF标准时出现的问题,并讨论未来的研究领域。