• 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.

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