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Primary Sclerosing Cholangitis — RACP Adult Medicine MCQ

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ModerateGastroenterologyPrimary Sclerosing CholangitisRACP Adult Medicine

A 30-year-old man with ulcerative colitis presents with raised ALP (380 U/L), GGT 290 U/L, and mildly elevated bilirubin. AMA is negative. p-ANCA is positive. MRCP shows multifocal intrahepatic and extrahepatic bile duct strictures with a beaded appearance. What is the most likely diagnosis?

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Correct answer: EPrimary sclerosing cholangitis

Cholestatic ALP with IBD (especially UC), negative AMA, positive p-ANCA, and MRCP showing multifocal bile duct strictures (beaded appearance) is primary sclerosing cholangitis. No proven medical therapy alters disease course (UDCA is controversial in PSC). Lifetime cholangiocarcinoma risk is 10-15%. Annual surveillance with MRCP ± CA19-9 is recommended. Liver transplant for end-stage disease.

Reference: EASL – 2022 – PSC Guidelines; eTG Gastrointestinal 2024