Abstract
Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease of unknown etiology and high mortality. Current therapeutic strategies have limited efficacy and the prognosis remains poor. Based on the histological observations of IPF lung tissues and experimental studies using lung fibrosis animal models, it is gradually accepted that impaired epithelial regeneration after lung injury is a critical mechanism underlying the pathogenesis of pulmonary fibrosis. The central role of AEC2 in this process has been well-elucidated, while the contribution of other lung progenitor/stem cells is less discussed. Recently, increasing studies have identified several non-AEC2 epithelial progenitor/stem cells with great plasticity to transform into mature AECs and reconstitute alveolar epithelium after lung injury. However, why these cells do not function as alternate stem cells to regenerate alveolar epithelium in IPF is still unknown. In this review, we discuss the contribution of lung epithelial progenitor/stem cells in the aberrant alveolar regeneration, and provide a novel perspective on the mechanism of IPF pathogenesis, in which non-AEC2 progenitors may play an essential role.
Highlights
It’s very likely that non-type II alveolar epithelial cells (AEC2) progenitor/stem cells participate in alveolar regeneration during progression of Idiopathic pulmonary fibrosis (IPF)
The potency of the non-AEC2 progenitor/stem cells to restore alveoli is almost only reported in mice under bleomycin treatment and/or influenza infection, which induce acute and severe lung injury
IPF is a progressive fibrotic disorder driven by the vicious cycle of abnormal epithelial injury/repair
Summary
Idiopathic pulmonary fibrosis (IPF) is the most common form of idiopathic interstitial pneumonia, with annual incidence of 1.7–27.1 cases per 100,000 persons (Raghu et al, 2006, 2016; Natsuizaka et al, 2014; Gjonbrataj et al, 2015; Harari et al, 2016, 2020; Hopkins et al, 2016; Strongman et al, 2018). Several studies have identified distal airway stem/progenitor cells referred to as DASCs, which expressed p63 and Krt5, and could regenerate the bronchiolar and alveolar epithelium after influenza or bleomycin injury in mice (Kumar et al, 2011; Zuo et al, 2015; Shi et al, 2019).
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