医学
特发性肺纤维化
肺纤维化
纤维化
任天堂
内科学
间质性肺病
寻常性间质性肺炎
作者
Paul J. Wolters,Timothy S. Blackwell,Oliver Eickelberg,James E. Loyd,Naftali Kaminski,Gisli Jenkins,Toby M. Maher,María Molina-Molina,Paul W. Noble,Ganesh Raghu,Luca Richeldi,Marvin I. Schwarz,Moisés Selman,Wim Wuyts,David A. Schwartz
标识
DOI:10.1016/s2213-2600(18)30007-9
摘要
Idiopathic pulmonary fibrosis (IPF) is a progressive, irreversible, and typically fatal lung disease characterised by subpleural fibrosis, subepithelial fibroblast foci, and microscopic honeycombing. Although understanding of the pathogenic mechanisms continues to evolve, evidence indicates that distal airway and alveolar epithelial cells are central drivers of the disease. In this Viewpoint, we review the history of naming and classifications used to define the disease now referred to as IPF, in the context of understanding the clinical presentation, causes, and pathogenesis of the disease. We aim to generate discussion on whether, given the substantial progress made in understanding the clinical, genetic, cellular, and molecular mechanisms involved in the development of IPF, a change of name should be considered. To initiate this discussion, we offer new suggestions to update the name of this disease and new approaches to classify all forms of pulmonary fibrosis.
科研通智能强力驱动
Strongly Powered by AbleSci AI