A 45-year-old woman presents with jaundice, pruritus, and fatigue for 6 months. ALP is 5× upper limit of normal with proportionally less elevated ALT/AST. Anti-mitochondrial antibody (AMA) is positive at 1:640 titre. Liver biopsy shows granulomatous destruction of medium-sized bile ducts with lymphocytic infiltrate. What is the first-line treatment?
- A Prednisolone 40 mg daily
- B Cholestyramine alone for symptomatic relief
- C Methotrexate as immunosuppressive therapy
- D Ursodeoxycholic acid (UDCA) 13–15 mg/kg/day ✓
Explanation
Primary biliary cholangitis (PBC) is characterized by positive AMA (M2 subtype targeting E2 component of pyruvate dehydrogenase complex), cholestatic LFTs, and granulomatous duct destruction. First-line treatment is UDCA (13–15 mg/kg/day), which improves liver biochemistry, delays histological progression, and reduces the need for liver transplantation (RCTs over 2 decades). About 40% of patients have an incomplete biochemical response to UDCA (defined by Paris I/II criteria) and may benefit from obeticholic acid (FXR agonist) as add-on therapy. Corticosteroids are not effective and may worsen bone disease.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.