The treatment of interstitial lung diseases, including idiopathic pulmonary fibrosis (IPF), remains challenging as current available antifibrotic agents are not effective in halting disease progression. Connective tissue growth factor (CTGF), also known as cellular communication factor 2 (CCN2), is a member of the CCN family of proteins that regulates cell signaling through cell surface receptors such as integrins, the activity of cytokines/growth factors, and the turnover of extracellular matrix (ECM) proteins. Accumulating evidence indicates that CTGF plays a crucial role in promoting lung fibrosis through multiple processes, including inducing transdifferentiation of fibroblasts to myofibroblasts, epithelial-mesenchymal transition (EMT), and cooperating with other fibrotic mediators such as TGF-β. Increased expression of CTGF has been observed in fibrotic lungs and inhibiting CTGF signaling has been shown to suppress lung fibrosis in several animal models. Thus, the CTGF signaling pathway is emerging as a potential therapeutic target in IPF and other pulmonary fibrotic conditions. This review provides a comprehensive overview of the current evidence on the pathogenic role of CTGF in pulmonary fibrosis and discusses the current therapeutic agents targeting CTGF using a systematic review approach.

间质性肺疾病的治疗，包括特发性肺纤维化 (IPF)，仍然具有挑战性，因为目前可用的抗纤维化药物无法有效阻止疾病进展。 结缔组织生长因子 (CTGF)，也称为细胞通讯因子 2 (CCN2)，是 CCN 蛋白家族 通过细胞表面受体调节细胞信号传导，例如整合素、细胞因子/生长因子的活性以及细胞外基质的周转（ ECM）蛋白质。越来越多的证据表明，CTGF 通过多个过程在促进肺纤维化中发挥着至关重要的作用，包括诱导成纤维细胞向肌成纤维细胞的转分化、上皮间质转化（EMT）以及与其他纤维化介质（如转化生长因子-β。在纤维化肺中观察到 CTGF 表达增加，并且在多种动物模型中抑制 CTGF 信号传导已被证明可以抑制肺纤维化。因此，CTGF 信号通路正在成为 IPF 和其他肺纤维化疾病的潜在治疗靶点。本综述全面概述了 CTGF 在肺纤维化中致病作用的现有证据，并采用系统评价方法讨论了当前针对 CTGF 的治疗药物。