- Cholestatic liver disease causing continued destruction of the bile ducts, cirrhosis, and end-stage liver disease.
- Predominantly affects young and middle-aged men, often with underlying inflammatory bowel disease.
- Patients may be asymptomatic at diagnosis but develop symptoms of pruritus and jaundice.
- Diagnosis involves laboratory tests and cholangiography.
- No effective medical therapy is available.
- Liver transplantation is the only treatment option for patients with advanced disease.
- Liver failure and cholangiocarcinoma are the leading causes of death in these patients.