Idiopathic pulmonary fibrosis (IPF) is a fatal interstitial lung disease with limited therapeutic options. Current evidence suggests that IPF may be initiated by repeated epithelial injuries in the distal lung, which are followed by abnormal wound healing responses that occur because of intrinsic and extrinsic factors. Mechanisms contributing to chronic damage of the alveolar epithelium in IPF include dysregulated cellular processes such as apoptosis, senescence, abnormal activation of the developmental pathways, aging, and genetic mutations. Therefore, targeting the regenerative capacity of the lung epithelium is an attractive approach in the development of novel therapies for IPF. Endogenous lung regeneration is a complex process involving coordinated cross-talk among multiple cell types and reestablishment of a normal extracellular matrix environment. This review will describe the current knowledge of reparative epithelial progenitor cells in the alveolar region of the lung and discuss potential novel therapeutic approaches for IPF, focusing on endogenous alveolar repair.

特发性肺纤维化 (IPF) 是一种致命的间质性肺病，治疗选择有限。目前的证据表明，IPF 可能是由远端肺的反复上皮损伤引发的，随后是由于内在和外在因素导致的异常伤口愈合反应。导致 IPF 中肺泡上皮慢性损伤的机制包括失调的细胞过程，如细胞凋亡、衰老、发育途径的异常激活、衰老和基因突变。因此，针对肺上皮的再生能力是开发 IPF 新疗法的一种有吸引力的方法。内源性肺再生是一个复杂的过程，涉及多种细胞类型之间的协调串扰和正常细胞外基质环境的重建。本综述将描述肺泡区修复性上皮祖细胞的当前知识，并讨论 IPF 的潜在新治疗方法，重点是内源性肺泡修复。