Sunny Sandhu, Aparna Goel
Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases that have undergone notable therapeutic advances in recent years. Recent advances in PBC have expanded treatment options beyond ursodeoxycholic acid, enabling earlier risk stratification and use of second-line therapies, allowing practitioners to personalize treatment. Meanwhile, multiple investigational agents are under evaluation in clinical trials for PSC, yet there remain no approved treatments. This review summarizes advances in epidemiology, diagnosis, and treatment of the underlying disease along with the extrahepatic manifestations associated with PBC and PSC. PBC has entered a treat-to-target era, while PSC remains without approved disease-modifying therapy despite a rapidly expanding trial pipeline.