Zhao Jinyu, Yue Ping, Mi Ningning, Li Matu, Fu Wenkang, Zhang Xianzhuo, Gao Long, Bai Mingzhen, Tian Liang, Jiang Ningzu, Lu Yawen, Ma Haidong, Dong Chunlu, Zhang Yong, Zhang Hengwei, Zhang Jinduo, Ren Yanxian, Suzuki Azumi, Wong Peng F, Tanaka Kiyohito, Rerknimitr Rungsun, Junger Henrik H, Cheung Tan T, Melloul Emmanuel, Demartines Nicolas, Leung Joseph W, Yao Jia, Yuan Jinqiu, Lin Yanyan, Schlitt Hans J, Meng Wenbo
Department of General Surgery, The First Hospital of Lanzhou University, Lanzhou, Gansu, China.
Department of Gastroenterology, The First Hospital of Lanzhou University, Lanzhou, Gansu, China.
Med Rev (2021). 2024 Jul 1;4(4):326-365. doi: 10.1515/mr-2024-0029. eCollection 2024 Aug.
Fibrosis resulting from pathological repair secondary to recurrent or persistent tissue damage often leads to organ failure and mortality. Biliary fibrosis is a crucial but easily neglected pathological feature in hepatobiliary disorders, which may promote the development and progression of benign and malignant biliary diseases through pathological healing mechanisms secondary to biliary tract injuries. Elucidating the etiology and pathogenesis of biliary fibrosis is beneficial to the prevention and treatment of biliary diseases. In this review, we emphasized the importance of biliary fibrosis in cholangiopathies and summarized the clinical manifestations, epidemiology, and aberrant cellular composition involving the biliary ductules, cholangiocytes, immune system, fibroblasts, and the microbiome. We also focused on pivotal signaling pathways and offered insights into ongoing clinical trials and proposing a strategic approach for managing biliary fibrosis-related cholangiopathies. This review will offer a comprehensive perspective on biliary fibrosis and provide an important reference for future mechanism research and innovative therapy to prevent or reverse fibrosis.
由反复或持续的组织损伤继发的病理性修复所导致的纤维化，常常会引发器官衰竭和死亡。胆汁性纤维化是肝胆疾病中一个关键但容易被忽视的病理特征，它可能通过胆道损伤继发的病理愈合机制，促进良性和恶性胆道疾病的发展与进展。阐明胆汁性纤维化的病因和发病机制，有助于胆道疾病的防治。在本综述中，我们强调了胆汁性纤维化在胆管疾病中的重要性，并总结了其临床表现、流行病学以及涉及胆小管、胆管细胞、免疫系统、成纤维细胞和微生物群的异常细胞组成。我们还聚焦于关键信号通路，深入探讨了正在进行的临床试验，并提出了一种管理与胆汁性纤维化相关胆管疾病的策略方法。本综述将提供关于胆汁性纤维化的全面观点，并为未来预防或逆转纤维化的机制研究和创新疗法提供重要参考。
