Liver disease in erythropoietic protoporphyria: insights and implications for management

Some protoporphyrin is subsequently reabsorbed in an enterohepatic circulation. 10 Protoporphyrin-induced hepatotoxicity is a rare complication occurring in 1-5% of patients, for whom liver transplantation is often required. Since the first liver transplant for EPP in 1980, 11 more than 40 further liver transplants have been carried out as treatment for advanced liver disease in this condition. [...]better evidence is available on the optimal method for detecting liver disease in EPP, clinicians caring for patients with EPP should remain vigilant and make an individualised assessment of risk based on the factors listed above, rather than relying arbitrarily on a standard set of tests to determine the need for liver biopsy or for specific therapeutic interventions.