Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction.
Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction.
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DOI：
10.1097/maj.0b013e31821a9d8e
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发表时间：
2011-06
期刊：
影响因子：
--
通讯作者：
Blackwell TS
中科院分区：
文献类型：
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作者：
Zoz DF;Lawson WE;Blackwell TS
特发性肺纤维化（IPF）的特征是进行性呼吸困难、肺实质间质浸润和肺功能检查受限。IPF是最常见和最严重的特发性间质性肺炎（IIP），大多数个体进展为呼吸衰竭。多种证据显示肺泡上皮细胞（AEC）在疾病中的重要作用。我们目前的疾病模式是，在遗传或获得性功能障碍II型AEC表型存在下，持续或重复的损伤性刺激导致AEC损伤/凋亡增加，正常肺泡结构再生不足，以及异常肺修复和成纤维细胞活化，导致进行性纤维化。虽然肺泡上皮细胞异常修复中涉及的损伤事件和过程的性质还不清楚，但正在进行的研究为更好地了解AEC维持稳态或促进纤维化的机制提供了希望。这些策略可能有望开发IPF的新型治疗方法。
Idiopathic pulmonary fibrosis (IPF) is characterized by progressive dyspnea, interstitial infiltrates in lung parenchyma, and restriction on pulmonary function testing. IPF is the most common and severe of the idiopathic interstitial pneumonias (IIPs), with most individuals progressing to respiratory failure. Multiple lines of evidence reveal prominent roles for alveolar epithelial cells (AECs) in disease. Our current disease paradigm is that ongoing or repetitive injurious stimuli in the presence of a genetic or acquired dysfunctional type II AEC phenotype results in increased AEC injury/apoptosis, deficiencies in regeneration of normal alveolar structure, and aberrant lung repair and fibroblast activation, leading to progressive fibrosis. While the nature of injurious events and processes involved in aberrant repair of the alveolar epithelium are not well understood, ongoing investigations provide hope to better understand mechanisms by which AECs maintain homeostasis or contribute to fibrosis. These strategies may hold promise for developing novel treatment approaches for IPF.