Primary sclerosing cholangitis (PSC) is most closely associated with which inflammatory bowel disease, and the characteristic histological finding on liver biopsy is:

• A Crohn disease; granulomatous destruction of bile ducts
• B Ulcerative colitis; ductopenia without fibrosis (vanishing bile duct syndrome)
• C Ulcerative colitis; concentric periductal fibrosis ('onion skin' fibrosis) around intrahepatic bile ducts ✓
• D Crohn disease; fibro-obliterative cholangitis with dense lymphocytic infiltrate

Explanation

PSC is strongly associated with ulcerative colitis (~75% of PSC patients have UC). The characteristic histological finding is concentric periductal 'onion skin' fibrosis around intrahepatic bile ducts, ultimately leading to bile duct obliteration and ductopenia. MRCP/ERCP shows multifocal strictures and dilatations ('beaded' appearance) of intra- and extrahepatic bile ducts. PSC markedly increases the risk of cholangiocarcinoma. Primary biliary cholangitis (PBC) shows granulomatous destruction of bile ducts (positive AMA).

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.