@article {Selman2004507, author = {Selman, Mois{\'e}s and Pardo, Annie}， title ={当事情出错时:探索间质性肺疾病进行性纤维化表型的可能机制}，位置-id ={2004507}，年= {2021}，doi ={10.1181 /13993003.04507-2020}，出版者={欧洲呼吸学会}，188bet官网地址肺间质性疾病(ILD)包括一大类已知和未知病因的异质性疾病，其特征是肺实质的弥漫性损害。在过去的几年里，不同类型的肺间质性肺病患者有发展进行性肺纤维化的风险，称为肺纤维化性肺纤维化(PF-ILD)。这是一种与特发性肺纤维化相似的表型，特发性肺纤维化是进行性纤维化的典型例子。PF-ILD不是一个独特的临床实体，但描述了一组具有相似临床行为的ILD。这种表型可能发生在疾病显示不同的病因和不同的生物病理学在他们的启动和发展。重要的是，这些实体可能在进入进行性纤维化阶段之前有改善或稳定的潜力。关键的问题是:(1)为什么即使经过适当的治疗，一部分患者也会发展为进展性和不可逆的纤维化表型;(2)驱动进展的致病机制可能是什么。在这里，我们提供了一个框架，突出了潜在的进展机制，包括遗传易感性、衰老、表观遗传学、结构纤维化扭曲、细胞外基质的异常组成和刚度，以及不同的促纤维化细胞亚群的出现。 Understanding the cellular and molecular mechanisms behind PF-ILD will provide the basis for identifying risk factors and appropriate therapeutical strategies.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr. Kohli has nothing to disclose.Conflict of interest: Dr. Selman reports personal fees from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Pardo has nothing to disclose.}, issn = {0903-1936}, URL = {//www.qdcxjkg.com/content/early/2021/01/28/13993003.04507-2020}, eprint = {//www.qdcxjkg.com/content/early/2021/01/28/13993003.04507-2020.full.pdf}, journal = {European Respiratory Journal} }