A 35-year-old man with known ulcerative colitis for 8 years presents with pruritus and fatigue. Labs: ALP 420 U/L, GGT 380 U/L, ALT 78 U/L, total bilirubin 2.1 mg/dL, p-ANCA positive. MRCP shows multifocal strictures and dilatations of intrahepatic and extrahepatic bile ducts with a beaded appearance. What is the diagnosis?

• A Primary biliary cholangitis
• B IgG4-related sclerosing cholangitis
• C Drug-induced cholestatic liver injury
• D Primary sclerosing cholangitis ✓

Explanation

Primary sclerosing cholangitis (PSC) classically presents with cholestatic liver enzyme elevation in a young man, frequently with coexisting inflammatory bowel disease (especially ulcerative colitis). MRCP showing multifocal bile duct strictures with intervening dilatations producing a beaded appearance is pathognomonic. p-ANCA is positive in 60-80% of cases. PBC shows anti-mitochondrial antibodies and affects small intrahepatic ducts. IgG4-related disease shows elevated serum IgG4 and responds to steroids.

Reference: Bailey and Love's Short Practice of Surgery, 28th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.