Primary Sclerosing Cholangitis: Diagnosis, Management, and Clinical Challenges

Primary sclerosing cholangitis is a rare, chronic, inflammatory disease of the biliary tree that leads to progressive ductal obliteration, hepatic dysfunction, and ultimately liver cirrhosis. Most patients eventually require liver transplantation or develop serious complications, the most severe being end-stage liver disease and cholangiocarcinoma. The global prevalence of PSC is rising and has been reported to reach up to 31.7 cases per 100,000 individuals, representing a significant challenge in both diagnosis and management. In this review, we aim to provide a clinically oriented overview of the diagnosis and management of PSC. Furthermore, we seek to highlight key challenges and areas of uncertainty that clinicians encounter in the diagnosis and treatment of patients with this complex disease.