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

Biliary fibrosis, a critical yet often overlooked feature in hepatobiliary disorders, contributes to the progression of both benign and malignant biliary diseases through pathological healing mechanisms following biliary tract injuries. Understanding its etiology and pathogenesis is key to preventing and treating these diseases. This review highlights the significance of biliary fibrosis in cholangiopathies, detailing clinical manifestations, epidemiology, and the roles of biliary ductules, cholangiocytes, immune system, fibroblasts, and microbiome. It also examines key signaling pathways and discusses ongoing clinical trials, proposing strategies for managing biliary fibrosis-related cholangiopathies. The review aims to guide future research and therapy development to prevent or reverse fibrosis.