A 32-year-old man with ulcerative colitis for 8 years develops progressive fatigue and pruritus. ALP 480 U/L, bilirubin 1.8 mg/dL, ALT 90 U/L. Antimitochondrial antibody is negative. MRCP shows multifocal short strictures of intrahepatic and extrahepatic bile ducts with intervening dilatation. What is the most likely diagnosis?

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

Explanation

Primary sclerosing cholangitis classically affects young men, is strongly associated with inflammatory bowel disease, produces cholestatic biochemistry, and shows multifocal strictures with beading of both intrahepatic and extrahepatic ducts on MRCP. Primary biliary cholangitis affects middle-aged women, is AMA positive, and spares the extrahepatic ducts. IgG4-related disease causes a dominant stricture rather than diffuse beading and responds to steroids. Stones cause a single obstructing lesion, not multifocal beading.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.