Idiopathic pulmonary fibrosis: new insights in its pathogenesis
Idiopathic pulmonary fibrosis: new insights in its pathogenesis
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DOI：
10.1016/s1357-2725(02)00091-2
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发表时间：
2002-12-01
影响因子：
4
通讯作者：
Selman, M
中科院分区：
文献类型：
--
作者：
Pardo, A;Selman, M
特发性肺纤维化（IPF）是一种病因不明的独特类型的慢性纤维化肺病。致病机制的顺序尚不清楚，但该疾病的特征是上皮损伤和活化，形成独特的上皮下成纤维细胞/肌成纤维细胞灶，以及过量的细胞外。基质积累这些病理过程通常导致肺结构的进行性和不可逆的变化，导致进行性呼吸功能不全和在相对短的时间内几乎普遍的终末结局。虽然研究主要集中在引发纤维化反应的炎症机制上，但最近的证据强烈表明肺泡上皮的破坏是潜在的致病事件。尽管迄今为止的治疗已被证明基本无效，但这种新方法开辟了几条有希望的治疗途径。(C)2002爱思唯尔科技有限公司。保留所有权利。
Idiopathic pulmonary fibrosis (IPF) is a unique type of chronic fibrosing lung disease of unknown etiology. The sequence of the pathogenic mechanisms is unknown, but the disease is characterized by epithelial injury and activation, the formation of distinctive subepithelial fibroblast/myofibroblast foci, and excessive extracellular. matrix accumulation. These pathological processes usually lead to progressive and irreversible changes in the lung architecture resulting in progressive respiratory insufficiency and an almost universally terminal outcome in a relatively short period of time. While research has largely focused on inflammatory mechanisms for initiating the fibrotic response, recent evidence strongly suggests that disruption of the alveolar epithelium is an underlying pathogenic event. Although treatment to date has proved largely ineffective, this new approach has opened up several promising therapeutic avenues. (C) 2002 Elsevier Science Ltd. All rights reserved.