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Primary sclerosing cholangitis — ABIM Board MCQ

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ModerateGastroenterology/HepatologyPrimary sclerosing cholangitisABIM Board

A 37-year-old with ulcerative colitis has fatigue, pruritus and cholestatic liver tests. MRCP shows multifocal strictures with intervening dilatation of intrahepatic and extrahepatic bile ducts. What is the most likely diagnosis?

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Correct answer: C — Primary sclerosing cholangitis affecting intrahepatic and extrahepatic ducts

The best answer is “Primary sclerosing cholangitis affecting intrahepatic and extrahepatic ducts”. The inflammatory-bowel-disease association and beaded multifocal intrahepatic and extrahepatic strictures are characteristic of PSC. Primary biliary cholangitis primarily affects small intrahepatic ducts and is associated with antimitochondrial antibodies, while a stone causes focal rather than diffuse multifocal obstruction.

Reference: AASLD Practice Guidance on Primary Sclerosing Cholangitis: https://www.aasld.org/practice-guidelines/primary-sclerosing-cholangitis-and-cholangiocarcinoma