若干细胞在特发性肺纤维化（IPF）发病机制中的作用.docVIP

若干细胞在特发性肺纤维化（IPF）发病机制中的作用* 徐飞 董竞成* （1.复旦大学附属华山医院中西医结合科2.复旦大学中西医结合研究所，上海，200040 ） 【摘要】特发性肺纤维化（Idiopathic Pulmonary Fibrosis, IPF）是间质性肺疾病中最常见的一种，主要发生在老年人，病变局限于肺部，组织病理学和（或）HRCT通常表现为寻常型间质性肺炎，其病死率高，预后差，但其病因不明，发病机制已从炎症假说演变为肺泡上皮过度修复假说。目前认为，IPF是一种多种诱发因素和途径共同作用的疾病。无论是细胞因子、生长因子、趋化因子、信号通路还是基因表型等等，其最终均作用于细胞而诱发形成IPF，本文将试图从细胞学角度阐述IPF的发病机理。 【关键词】IPF；发病机制；细胞学水平；间质性肺疾病 A Few Cells in The Pathogenesis of Idiopathic Pulmonary Fibrosis (IPF) 【Abstract】IPF is one of the most common interstitial lung disease，mainly in the elderly, with high?mortality,poor prognosis and unknown etiology. Pathological changes limit to the lungs, and histopathology and (or) HRCT are typically characterized by UIP. Pathogenesis has changed from excessive inflammation hypothesis to alveolar epithelial repair hypothesis. HYPERLINK ../../../Users/lenovo/AppData/Local/Yodao/DeskDict/frame/20150403224618/javascript:void(0);At?HYPERLINK ../../../Users/lenovo/AppData/Local/Yodao/DeskDict/frame/20150403224618/javascript:void(0);present, IPF is considered as a disease with a variety of factors and pathways. Cytokines, growth factors, chemokines, signaling pathways, or genetic phenotypes and so on are all involved in the process. And they ultimately act on cells and then induce formation of IPF. This article will elaborate the pathogenesis of IPF from cellular level. 【Key words】IPF; pathogenesis; cells; ILD Xu Fei Dong Jingcheng (1.Integrated department of Chinese and western medicine, Huashan hospital, FD University 2.Institute of Integrated Traditional and Western Medicine of FD DAN University) IPF(Idiopathic Pulmonary Fibrosis, IPF)为原因不明、出现在成人、局限于肺、进行性致纤维化的最常见的间质性肺疾病，其组织病理学和放射学常常表现为寻常型间质性肺炎(usual interstitial pneumonia，UIP) ADDIN NE.Ref.{626F83E4-3E05-4F4E-BBC4-AEA9B7D9E971}[1]，有其特征性的临床、影像学、病理学表现。尽管目前对其确切病因认识尚不明确，但吸烟、耕作、职业病、病毒和细菌感染都是易感因素。IPF是间质性肺疾病(Interstitial Lung Disease,ILD)中最常见和最严重的类型之一，预后差，死亡率比许多癌症还要高，确诊后平均50%的患者存活3年，5年生存率仅为20%左右 ADDIN NE.Ref.{20DA019A-C01E-4831-980AC19}[2]。据报道美国IPF发病率和患病率分别为