Primary Sclerosing Cholangitis

• Idiopathic disorder characterized by progressive inflammation and fibrosis → strictures of extrahepatic and intrahepatic bile ducts.
• Associated with ulcerative colitis (often in young men) and ↑ risk of cholangiocarcinoma

Signs and Symptoms

• Progressive jaundice, pruritus and fatigue

Investigation

• Bloods: ↑ ALP, ↑ bilirubin, p-ANCA positive
• MRCP/ERCP: multiple bile duct strictures and dilatations (beading)
• Liver biopsy: periductal sclerosis (onion skinning)
• Endoscopy: may show ulcerative colitis (PSC – Please Screen the Colon!)

Management

• ERCP with dilatation and stenting of strictures
• Liver transplantation
• Ursodeoxycholic acid has been shown to improve liver function in select patients

Reference: First-Aid for the USMLE Step 2 (10th Edition)