Abstract:Primary biliary cholangitis ( PBC) is a prevalent autoimmune disease in middle-aged women,characterized by immune-mediated lymphocyte infiltration and bile duct destruction in intrahepatic bile ducts. The pathogenesis of PBC is complex, and its etiology remains unclear. Animal models can help to elucidate the pathogenesis and clinical treatment of PBC, but cannot fully simulate its clinical manifestations and pathological changes, even if the models show similar features of disease progression to those in humans. Here, we systematically compared the heterogeneity of PBC mouse models constructed using different induction method in terms of their genetic background, modeling principles, pathogenic cells, and immune characteristics. We then reviewed the differences in immune regulation and disease phenotypes among different PBC mouse models and explored the preclinical treatment strategies based on PBC mouse models. This work contributes to our understanding and the optimization of PBC mouse models, further elucidating the complex pathological processes of PBC and providing new insights for precise therapeutic interventions.