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 - Knipe,瑞秋s Y1 - 2020/01/01 UR - //www.qdcxjkg.com/content/early/2020/04/01/13993003.00100 - 2019. -抽象N2 -特发性肺纤维化(IPF)被认为是由于异常的组织修复过程中应对慢性或重复的肺损伤。受伤的起源和性质,以及它的细胞和分子的目标,可能是异构的,复杂疾病的准确的临床前模型,使治疗目标的挑战。工作正在进行中,以识别中央通道纤维发生可能允许针对异常修复过程无论初始损伤刺激。特异表达内皮通透性和血管渗漏一直是研究急性肺损伤和修复作用。证据表明,这些过程重要性的肺纤维化疾病的发病机理正在增长。在non-fibrosing肺部疾病内皮通透性增加,但它自限性的方式解决条件,如细菌性肺炎和急性呼吸窘迫综合征(ARDS)。在进步fibrosing IPF等疾病,渗透率似乎持续不过也可以预测死亡率。在这个存活率存在审查中,我们总结数据在IPF内皮通透性的作用,关注持续的有害后果内皮研究,以应对在肺部炎症和纤维化。我们建议持续的渗透率和肺部血管泄漏有可能建立和扩大pro-fibrotic环境。治疗性干预旨在识别和“堵”的泄漏可能因此显著有利于防止从肺损伤过渡到肝纤维化、应该为未来的研究领域。FootnotesThis手稿最近发表在《欧洲呼吸杂志》上。 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 -