date: “2024-01-01” title: Rx-Primary biliary cholangitis PBC alias: rx-PBC
Rx-Primary biliary cholangitis (PBC)
- Obeticholic acid (FXR agonist): monoRx if cannot tolerate UDCA (but not in decompen cirrhosis) or if no Δ w/ UDCA after 1 y; found to ↓ Aφ, ↑ pruritus (NEJM 2016;375:631)
- ursodeoxycholic acid (UDCA) (13-15 mg/kg bid), monitor for 3-6 mos → ~25% complete response, ↑ survival & ↓ histologic change & ↓ complications (varices). Biochemical response predicts clinical outcome.
- Bezafibrate (not available in U.S. but fenofibrate similar) appears to be effective 2nd-line agent in combo w/ UDCA if inadequate response to UDCA (NEJM 2018;378:2171)
- Pruritus: cholestyramine (give 2-4 h after UDCA); if refractory sx: naltrexone, rifampin
- If ESLD: liver tx; ~20% recur but no impact on long-term survival