Medicine · Liver Disease (Cirrhosis, Hepatitis, Autoimmune, Wilson's, Hemochromatosis)

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
Correct answer: C. AMA-negative primary biliary cholangitis

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.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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