A 40-year-old woman presents with fatigue, pruritus, and elevated alkaline phosphatase. ANA and anti-smooth muscle antibodies are negative. Anti-mitochondrial antibodies (AMA) are strongly positive. Liver biopsy shows florid duct lesion with granuloma formation around the interlobular bile ducts and a lymphoplasmacytic infiltrate. The diagnosis is:

• A Primary sclerosing cholangitis
• B Autoimmune hepatitis
• C Drug-induced cholestatic hepatitis
• D Primary biliary cholangitis (PBC) ✓

Explanation

Primary biliary cholangitis (PBC, formerly PBC of the liver) is an autoimmune disease predominantly affecting middle-aged women, characterized by positive AMA (anti-PDC-E2 in 95%), elevated ALP, and florid duct lesions with granulomatous inflammation attacking interlobular bile ducts. The four histological stages progress from portal inflammation and duct damage (Stage I) through ductopenia and fibrosis (Stage II–III) to cirrhosis (Stage IV). Primary sclerosing cholangitis (PSC) is AMA-negative, predominantly affects young men with IBD, and shows 'onion skin' periductal fibrosis of large ducts.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.