Curriculum · Gastroenterology
Primary sclerosing cholangitis
What it is
Primary sclerosing cholangitis is a chronic cholestatic liver disease of unknown cause, characterized by progressive bile duct destruction and biliary cirrhosis. The cholestasis leads to progressive destruction of both intrahepatic and extrahepatic bile ducts, and it is a cholestatic liver disease of the medium and large bile ducts, not of the small bile ducts as in primary biliary cholangitis. The demographic is given differently in different places: the patient is described as a middle-aged man in one source and as men aged 20 to 30 years in another. Both make it a disease of men.
Causes and risk
The cause is unknown. The association is with IBD: eighty percent of patients have an IBD, most often ulcerative colitis.
How it presents
Fatigue and pruritus come first, and obstructive jaundice later. Characteristic findings are pruritus or jaundice, an elevated serum alkaline phosphatase level, an elevated total bilirubin level, and modestly elevated AST and ALT levels. The liver tests show a cholestatic picture, with elevated ALP, GGT and direct bilirubin.