Liver disease in erythropoietic protoporphyria: insights and implications for management
Liver disease in EPP
Pathogenesis of liver disease in EPP
- Mild liver disease;
- Deteriorating liver disease; and
- Terminal phase of EPP‐associated liver disease.
Mild liver disease
Deteriorating liver disease
- To increase the excretion of protoporphyrin into bile by the oral administration of the bile salts chenodeoxycholic acid50,52 or ursodeoxycholic acid.41
- To reduce protoporphyrin production by suppressing erythropoiesis using iron,53,54 red cell transfusions55 or infusion of haematin,56,65 all of which are intended to reduce the drive for haem synthesis.
- To reduce the pool of circulating plasma protoporphyrin by plasmapheresis,57,58 haemodialysis,59and exchange transfusions.55,60
- To reduce protoporphyrin levels by interrupting the enterohepatic circulation with administration of cholestyramine59,61 and activated charcoal.54,62
- To reverse oxidative stress in EPP by intravenous vitamin E therapy.63