A 35-year-old man with a 10-year history of ulcerative colitis develops progressive pruritus and fatigue. Examination is unremarkable except for excoriations. ALP is 420 U/L, bilirubin 1.4 mg/dL, ALT mildly elevated. MR cholangiography shows multifocal short strictures of both intrahepatic and extrahepatic ducts with intervening dilatation. Anti-mitochondrial antibody is negative. What is the most likely diagnosis?

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

Explanation

Multifocal strictures with beading of both intrahepatic and extrahepatic bile ducts on cholangiography, in a cholestatic young man with ulcerative colitis, defines primary sclerosing cholangitis; about 80 percent of cases are associated with inflammatory bowel disease. Primary biliary cholangitis affects middle-aged women, targets small intrahepatic ducts, and gives positive antimitochondrial antibody. IgG4-related disease produces a dominant stricture and mass rather than diffuse beading.

Reference: Sleisenger and Fordtran's Gastrointestinal and Liver Disease, 11th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.