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AIH-PBC Overlap — RACP Adult Medicine MCQ

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HardGastroenterologyAIH-PBC OverlapRACP Adult Medicine

A 50-year-old woman presents with fatigue, pruritus, and cholestatic LFTs (ALP 480 U/L, GGT 350 U/L, ALT 120 U/L). AMA is positive (1:640). IgG is elevated (22 g/L), ANA positive (1:320). Liver biopsy shows florid duct lesion AND interface hepatitis with plasma cells. MRCP shows normal bile ducts. What is the most likely diagnosis?

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Correct answer: A — AIH-PBC overlap syndrome

Features of both PBC (AMA positive, cholestatic LFTs, florid duct lesion) AND AIH (elevated IgG, ANA positive, interface hepatitis with plasma cells) meeting Paris criteria for both components is AIH-PBC overlap syndrome. Affects ~10% of PBC patients. Treatment: UDCA (for PBC component) PLUS immunosuppression (prednisolone ± azathioprine for AIH component).

Reference: EASL – 2025 – AIH; EASL 2022 PBC; Paris Criteria