Resumen

• 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.