2025 年第 5 期 第 20 卷

特发性肺纤维化发病机制相关信号通路的研究现状

Research status of signaling pathways related to the pathogenesis of idiopathic pulmonary fibrosis

作者：张礼鹏王晶

英文作者：Zhang Lipeng Wang Jing

单位：海南医科大学第二附属医院呼吸内科，海口570000

英文单位：Department of Respiratory Medicine the Second Affiliated Hospital of Hainan Medical University Haikou 570000 China

关键词：特发性肺纤维化；发病机制；信号通路；研究现状

英文关键词：Idiopathicpulmonaryfibrosis;Pathogenesis;Signalingpathways;Researchstatus

• 摘要：

• 特发性肺纤维化（IPF）是一种致命性肺部疾病，特征为肺组织进行性纤维化，且其发病机制尚不完全明了。近年来的研究发现，多种信号通路在IPF的发生和进展中起着关键作用，包括转化生长因子β、 Wnt/β-连环蛋白、丝裂原活化蛋白激酶、磷脂酰肌醇-3-激酶/蛋白激酶B、Rho/Rho相关激酶、Janus蛋白酪氨酸激酶/信号转导和转录活化蛋白、哺乳动物雷帕霉素靶蛋白等。这些通路通过增强成纤维细胞的增殖、分化及细胞外基质的沉积，推动肺间质纤维化的进程。然而，许多上游和下游信号分子尚未明确。此外，血管内皮生长因子、成纤维细胞生长因子及血小板源性生长因子等细胞因子也在IPF的发生发展中发挥重要作用。深入理解这些信号通路及其相互作用有助于为IPF的早期诊断和治疗提供新的靶点和策略。本文旨在总结当前的研究进展，并探讨未来的研究方向，以期为IPF的临床管理提供科学依据。

• Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease characterized by progressive fibrosis of lung tissue, and its pathogenesis is not fully understood. Recent studies have found that a variety of signaling pathways play a key role in the occurrence and progression of IPF. Including transforming growth factor β, Wnt/β-catenin, mitogen-activated protein kinase, phosphatidylinositol 3 kinase/protein kinase B, Rho/ Rho-associated kinase, Janus kinase/signal transducer and activator of transcription protein, mammalian target of rapamycin, etc. These pathways promote the process of pulmonary interstitial fibrosis by enhancing the proliferation, differentiation and extracellular matrix deposition of fibroblasts. However, many upstream and downstream signaling molecules have yet to be defined. In addition, cytokines such as vascular endothelial growth factor, fibroblast growth factor and platelet-derived growth factor also play an important role in the occurrence and development of IPF. An in-depth understanding of these signaling pathways and their interactions will help to provide new targets and strategies for early diagnosis and treatment of IPF. This article aims to summarize the current research progress and explore future research directions in order to provide a scientific basis for the clinical management of IPF.