Idiopathic pulmonary fibrosis (IPF) is a serious life-threatening lung disease, and the median survival period of PF patients after diagnosis is only 2.5–3.5 years. At present, there are no effective drugs or therapeutics to reverse or even inhibit IPF. The main pathological characteristics of pulmonary fibrosis (PF) include damage to alveolar epithelial cells, fibroblast activation and extracellular matrix accumulation, which gradually lead to damage to the lung structure and decreased lung function. It is important to understand the cellular and molecular mechanisms of PF comprehensively and clearly. In this paper, critical signaling pathways related to PF were reviewed to present updates on the molecular mechanisms of PF.

特发性肺纤维化（IPF）是一种严重的威胁生命的肺部疾病，诊断后PF患者的中位生存期仅为2.5-3.5年。目前，尚无有效的药物或疗法可以逆转甚至抑制IPF。肺纤维化（PF）的主要病理特征包括肺泡上皮细胞受损，成纤维细胞活化和细胞外基质积聚，逐渐导致肺结构受损和肺功能下降。全面而清晰地了解PF的细胞和分子机制非常重要。在本文中，综述了与PF相关的关键信号通路，以介绍PF分子机制的最新进展。