A 30-year-old woman presents with fatigue, pruritus, and jaundice. Laboratory studies show total bilirubin 6.2 mg/dL (direct 4.8), ALP 520 IU/L, GGT 380 IU/L, ALT 95 IU/L, AST 82 IU/L. AMA is positive at 1:640. Liver biopsy shows florid duct lesions with granulomatous destruction of interlobular bile ducts. Which is the most likely diagnosis?
- A Primary sclerosing cholangitis
- B Primary biliary cholangitis ✓
- C Autoimmune hepatitis
- D Drug-induced liver injury
Correct answer: B. Primary biliary cholangitis
Explanation
Primary biliary cholangitis (PBC) is characterized by immune-mediated destruction of intrahepatic bile ducts, predominantly affecting middle-aged women. The hallmark is anti-mitochondrial antibody (AMA) positivity in 90-95% of cases. Biopsy shows florid duct lesions with granulomatous cholangitis. The cholestatic pattern (elevated ALP and GGT with mild transaminase elevation) is typical. Primary sclerosing cholangitis shows ANCA positivity and bile duct strictures on imaging. Autoimmune hepatitis shows elevated transaminases and ANA/SMA positivity.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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