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 the etiology is not yet clear. Animal models are meaningful for elucidating the pathogenesis and clinical treatment of PBC. Animal models cannot fully simulate the clinical manifestations and pathological changes of PBC, even the models show similar features of disease progression with human. Here, we systematically compare the heterogeneity of PBC mouse models constructed by different induction methods in terms of genetic background, modeling principles, pathogenic cells, and immune characteristics. Then, we review the differences in immune regulation and disease phenotypes among different PBC mouse models and preliminarily explore preclinical treatment strategies based on PBC mouse models. This work contributes to understanding and optimizing PBC mouse models, further elucidating the complex pathological processes of PBC, and providing new insights for the precise therapeutic intervention of PBC.