Primary Sclerosing Cholangitis — RACP Adult Medicine MCQ
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Correct answer: E — Primary 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