Medical Review (Aug 2024)

Biliary fibrosis is an important but neglected pathological feature in hepatobiliary disorders: from molecular mechanisms to clinical implications

  • 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

DOI
https://doi.org/10.1515/mr-2024-0029
Journal volume & issue
Vol. 4, no. 4
pp. 326 – 365

Abstract

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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.

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