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 hepa...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.展开更多
基金supported by the National Key Research and Development Program of China (2022YFC2407405)the Joint Research Fund Major Project of Gansu Province (23JRRA1488)the Medical Innovation and Development Project of Lanzhou University (lzuyxcx-2022-157).
文摘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.
