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AIH-PSC Overlap — RACP Adult Medicine MCQ

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HardGastroenterologyAIH-PSC OverlapRACP Adult Medicine

A 25-year-old man with ulcerative colitis presents with elevated ALP (350 U/L) and ALT (180 U/L). AMA is negative. ANA is positive. IgG is elevated. MRCP shows multifocal intrahepatic bile duct strictures. Liver biopsy shows interface hepatitis with plasma cell infiltrate AND concentric periductal fibrosis (onion-skinning). What is the most likely diagnosis?

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Correct answer: A — AIH-PSC overlap syndrome

Features of PSC (IBD association, MRCP strictures, periductal fibrosis) AND AIH (elevated IgG, ANA positive, interface hepatitis with plasma cells) is AIH-PSC overlap syndrome. More common in children/young adults with IBD. Treatment: UDCA for the cholestatic component plus immunosuppression for the AIH component. Prognosis may be better than pure PSC if AIH component responds to treatment.

Reference: EASL – 2025 – AIH; EASL 2022 PSC