A 40-year-old woman presents with progressive jaundice, pruritus, and elevated ALP/GGT. Antimitochondrial antibodies (AMA) are strongly positive. Liver biopsy shows lymphocytic infiltration and granulomatous destruction of interlobular bile ducts. The diagnosis is:
- A Primary sclerosing cholangitis
- B Primary biliary cholangitis ✓
- C Autoimmune hepatitis
- D IgG4-related cholangiopathy
Explanation
Primary biliary cholangitis (PBC, formerly PBC cirrhosis) is characterized by autoimmune destruction of small intrahepatic bile ducts, mediated by CD4+ and CD8+ T lymphocytes sensitized to mitochondrial antigens. AMA (particularly anti-PDC-E2) are present in >95% of cases and are highly specific. Liver biopsy shows non-suppurative cholangitis with epithelioid granulomas around damaged bile ducts (florid duct lesion). It predominantly affects middle-aged women and leads to cholestatic cirrhosis if untreated. PSC preferentially affects large bile ducts, is AMA-negative, and shows 'onion-skin' periductal fibrosis.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.