TY - T1的纤维化肺血管通透性的JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.00100 -2019欧元SP - 1900100 AU Probst克莱门斯k . AU - Montesi悉尼b . AU -有所,本杰明·d . AU -谢伊,巴里·s . AU - KnipeN2 -特发性肺纤维化(Idiopathic Pulmonary Fibrosis, IPF)被认为是由于慢性或重复性肺损伤引起的异常组织修复过程。损伤的来源和性质，以及它的细胞和分子靶点，很可能是异构的，这使得疾病的精确临床前建模复杂化，并使治疗靶向成为一个挑战。研究人员正在努力确定纤维生成的中枢通路，这可能使得无论初始损伤刺激如何靶向异常修复过程。由于内皮细胞通透性和血管渗漏在急性肺损伤和修复中的作用，长期以来人们一直在研究它们。越来越多的证据表明，这些过程对纤维化肺疾病的发病机制是重要的。内皮细胞通透性在非纤维化肺疾病中增加，但在细菌性肺炎和急性呼吸窘迫综合征(ARDS)等情况下以自我限制的方式解决。在进行性纤维化疾病如IPF中，通透性似乎持续存在，并可能预测死亡率。在这篇生成假说的综述中，我们总结了有关内皮细胞通透性在IPF中的作用的现有数据，并重点讨论了持续的内皮细胞高通透性在应对肺部炎症和纤维化过程中的有害后果。我们认为，肺中持续的通透性和血管泄漏有可能建立和放大前纤维化环境。 Therapeutic interventions aimed at recognising and “plugging” the leak may therefore be of significant benefit for preventing the transition from lung injury to fibrosis and should be areas for future research.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: Probst has nothing to disclose.Conflict of interest: Dr. Montesi reports other from United Therapeutics Corporation, other from Promedior, outside the submitted work.Conflict of interest: Dr. Medoff has nothing to disclose.Conflict of interest: Dr. Shea reports personal fees from Genentech, personal fees from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Knipe has nothing to disclose. ER -