Figure 1.

Immune mechanism based therapeutic strategies in PBC. B-cell activating factor of the tumor necrosis factor family (BAFF) and CD20-targeted therapy play a crucial role in breaking immune tolerance and stimulating immune responses in primary biliary cholangitis (PBC). Promising novel therapeutic targets for PBC treatment are highlighted in red. One potential strategy is B-cell targeted therapy, including the use of anti-CD20, anti-BAFF, or a combination of both. Another approach is T-cell-directed immunotherapy, which involves inhibiting Th1 and Th17 cell differentiation by regulating related cytokines, up-regulating Treg function and number via chimeric antigen receptor-modified Tregs (CAR-Tregs). Additionally, interfering with costimulatory signals between cells, such as targeting CTLA-4, PD-1, and CD40, has shown potential in treating PBC. Regulating related cytokines, targeting chemokines, and inhibiting signal pathways involved in PBC pathogenesis, such as monoclonal antibodies against CXCL10, JAK inhibitors, or inhibitors of the NF-κB signal pathway, represent a fourth potential approach. Finally, mesenchymal stem cells (MSCs) can be used to regulate innate and adaptive immune responses by differentiating induced pluripotent stem cells. BEC, biliary epithelial cell; PBC, primary biliary cholangitis; BAFF, B-cell -activating factor; Th1, type 1 T helper cell; Th17, type 17 T helper cell; Treg, regulatory T cell; CAR, chimeric antigen receptor; CTLA-4,cytotoxic T-lymphocyte–antigen-4; PD-1, Programmed death-1; CXCL10, chemokine (C-X-C motif) ligand 10; MSC, mesenchymal stem cells.