Pathology · Hepatobiliary and Pancreatic Pathology

A 45-year-old woman with primary biliary cholangitis has a liver biopsy showing granulomatous destruction of interlobular bile ducts with a dense lymphocytic infiltrate. The serological marker most specific for this condition is:

  • A Anti-mitochondrial antibody (AMA), specifically against pyruvate dehydrogenase complex E2
  • B Anti-smooth muscle antibody (ASMA)
  • C Anti-liver-kidney microsomal antibody (anti-LKM1)
  • D Anti-neutrophil cytoplasmic antibody (p-ANCA)
Correct answer: A. Anti-mitochondrial antibody (AMA), specifically against pyruvate dehydrogenase complex E2

Explanation

Primary biliary cholangitis (PBC) is defined by anti-mitochondrial antibodies (AMA) in >95% of cases, specifically targeting the E2 component of pyruvate dehydrogenase complex on the inner mitochondrial membrane. The histological hallmark is florid duct lesion — granulomatous inflammation destroying interlobular bile ducts. ASMA is seen in autoimmune hepatitis type 1; anti-LKM1 in AIH type 2; p-ANCA in primary sclerosing cholangitis.

Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

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