Primary sclerosing cholangitis

- disorder of unknown cause characterised by non malignant non bacterial inflammation, fibrosis, and stricture of the intra and extrahepatic bile ducts
- associated with ulcerative colitis
- more common in men (70%) and average age of 40
- presents with fluctuating jaundice, pruritus and cholangitis
- positive p ANCA
- raised ALP initially followed by raised bilirubin, hypergammaglobulinaemia
- ERCP: multiple strictures in biliary system
- cholangiogram: intrahepatic and extrahepatic duct strictures ('beaded' appearance)
- 10% risk of cholangiocarcinoma
- treatment: ursodeoxycholic acid, liver transplantation (only proven long term treatment)