Department of Respiratory Medicine, Beijing Tiantan Hospital, Capital Medical University, No.119 South Fourth Ring West Road, Fengtai District, Beijing, 100070, People's Republic of China.
Department of Respiratory Medicine, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong An Road, Xicheng District, Beijing, 100050, People's Republic of China.
Lung. 2022 Oct;200(5):539-547. doi: 10.1007/s00408-022-00571-w. Epub 2022 Sep 22.
Idiopathic pulmonary fibrosis (IPF) is a progressive and irreversible pulmonary interstitial disease that seriously affects the patient's quality of life and lifespan. The pathogenesis of IPF has not been clarified, and its treatment is limited to pirfenidone and nintedanib, which only delays the decline of lung function. Alveolar epithelial type 2 (AT2) cells are indispensable in the regeneration and lung surfactant secretion of alveolar epithelial cells. Studies have shown that AT2 cell dysfunction initiates the occurrence and progression of IPF. This review expounds on the AT2 cell dysfunction in IPF, involving senescence, apoptosis, endoplasmic reticulum stress, mitochondrial damage, metabolic reprogramming, and the transitional state of AT2 cells. This article also briefly summarizes potential treatments targeting AT2 cell dysfunction.
特发性肺纤维化(IPF)是一种进行性和不可逆转的肺间质疾病,严重影响患者的生活质量和寿命。IPF 的发病机制尚未阐明,其治疗仅限于吡非尼酮和尼达尼布,只能延缓肺功能下降。肺泡上皮细胞 2 型(AT2)在肺泡上皮细胞的再生和肺表面活性物质分泌中不可或缺。研究表明,AT2 细胞功能障碍引发 IPF 的发生和进展。本文阐述了 IPF 中 AT2 细胞功能障碍,涉及衰老、凋亡、内质网应激、线粒体损伤、代谢重编程和 AT2 细胞的过渡状态。本文还简要总结了针对 AT2 细胞功能障碍的潜在治疗方法。