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A variety of receptors regulating bile duct metabolism, such as PPAR and farnesoid-X receptors (FXR), appear to play a crucial role in disease pathogenesis, making them attractive targets for disease-modifying drugs ( Figure 2 3 Available treatment options 3.1 Current treatment regimens 3.1.1 Ursodeoxycholic acid UDCA has been the cornerstone of PBC treatment since its approval in 1994