A 29-year-old man with ulcerative colitis develops progressive pruritus and fatigue. ALP is 420 U/L with normal transaminases. MRCP shows multifocal short segment strictures of both intrahepatic and extrahepatic ducts alternating with normal caliber segments, producing a beaded appearance. What is the most likely diagnosis?

• A Primary biliary cholangitis
• B Choledocholithiasis
• C IgG4-related sclerosing cholangitis
• D Primary sclerosing cholangitis ✓

Explanation

Multifocal biliary strictures with intervening saccular dilatation on cholangiography give the classic beading pattern of PSC, which affects young men and is strongly associated with inflammatory bowel disease, especially ulcerative colitis. PBC is a small duct disease of middle-aged women with antimitochondrial antibody and a normal cholangiogram, and IgG4-related sclerosing cholangitis produces a dominant stricture mimicking cholangiocarcinoma rather than diffuse beading.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.