A 42-year-old woman presents with fatigue, jaundice, pruritus, and elevated ALP (3× ULN). Liver biopsy shows granulomatous destruction of interlobular bile ducts. AMA is negative, but anti-gp210 antibody is positive. What is the most likely diagnosis?
- A Primary sclerosing cholangitis
- B Drug-induced cholestasis
- C AMA-negative primary biliary cholangitis ✓
- D Autoimmune hepatitis overlap syndrome
Explanation
The clinical picture with cholestasis, pruritus, and florid duct lesion on biopsy is classic for primary biliary cholangitis. About 5% of PBC patients are AMA-negative. Anti-gp210 and anti-sp100 antibodies are highly specific for PBC in this setting. PSC shows concentric fibrosis on cholangiography, not granulomatous duct destruction. AIH overlap would show interface hepatitis.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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