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Alveolar Epithelial Type 2 Cell Dysfunction in Idiopathic Pulmonary Fibrosis

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Abstract

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.

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Funding

This work was supported by the National Natural Science Foundation of China (Grant No. 82000065), Beijing Key Clinical Specialty Construction Project (2020–2022).

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  1. 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

    Weiwei Zhu & Jie Zhang

  2. Department of Respiratory Medicine, Beijing Friendship Hospital, Capital Medical University, No. 95 Yong An Road, Xicheng District, Beijing, 100050, People’s Republic of China

    Chunting Tan

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WZ conceived, wrote, and edited the manuscript. CT and JZ conceived and edited the manuscript. All authors read and approved the final manuscript.

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Zhu, W., Tan, C. & Zhang, J. Alveolar Epithelial Type 2 Cell Dysfunction in Idiopathic Pulmonary Fibrosis. Lung 200, 539–547 (2022). https://doi.org/10.1007/s00408-022-00571-w

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