Dept of Medicine, University of Washington, Seattle, WA, USA.
Center for Interstitial Lung Disease, University of Washington, Seattle, WA, USA.
Eur Respir J. 2019 Feb 21;53(2). doi: 10.1183/13993003.01699-2018. Print 2019 Feb.
Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease of unknown origin that is associated with high morbidity and mortality. In this perspective, we briefly review the current understanding of the pathophysiology of IPF and the importance of environmental triggers as a precipitant of disease. We discuss occult intrinsic and extrinsic environmental factors that affect the lung microenvironment and may contribute to the development and progression of disease. The clinical implications of this framework need to be further elucidated, because prompt identification and elimination of occult exposures may represent a novel treatment modality.
特发性肺纤维化（IPF）是一种病因不明的进行性肺纤维化疾病，其发病率和死亡率都很高。从这个角度来看，我们简要回顾了对 IPF 病理生理学的现有理解，以及环境诱因作为疾病诱发因素的重要性。我们讨论了影响肺微环境并可能导致疾病发展和进展的隐匿内在和外在环境因素。需要进一步阐明这一框架的临床意义，因为及时识别和消除隐匿暴露可能代表一种新的治疗方式。
