A 38-year-old man with ulcerative colitis develops progressive fatigue and pruritus. Alkaline phosphatase is 420 U/L with bilirubin 2 mg/dL. MR cholangiography shows multifocal short strictures of both intrahepatic ducts with intervening dilatation. The most likely diagnosis is:

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

Explanation

Multifocal biliary strictures with beading on MRCP, in a man with ulcerative colitis and cholestatic biochemistry, is classic for primary sclerosing cholangitis. PBC shows diffuse uniform duct injury without discrete strictures and affects middle-aged women with AMA positivity. Ischemic cholangiopathy follows hepatic artery injury or transplantation rather than an indolent course.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.