Current Treatment of Primary Biliary Cholangitis and Primary Sclerosing Cholangitis: A Comprehensive Review

Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC) are chronic cholestatic liver diseases characterized by bile duct injury that may progress to fibrosis, cirrhosis, liver failure, and malignancy and ultimately require liver transplantation. Although these diseases share several clinical features, they differ substantially in pathogenesis, clinical course, and therapeutic approaches. Ursodeoxycholic acid remains the standard first-line therapy for PBC; patients with an inadequate biochemical response may be treated with second-line agents such as seladelpar, elafibranor, or fibrates. In contrast, no approved therapy has been shown to slow disease progression in PSC. Emerging therapies primarily target symptom and complication management, and overall management focuses on surveillance and timely referral for liver transplantation. This review summarizes current approaches to risk assessment, treatment, symptom management, monitoring, and transplantation decision-making in both conditions. Cite this article as: Abadi-Ron V, Marenco-Flores A, Rojas-Amaris N, et al. Current treatment of primary biliary cholangitis and primary sclerosing cholangitis: A comprehensive review. Turk J Gastroenterol. 2026;37(8):824-838.