Liver transplantation for primary biliary cirrhosis and primary sclerosingcholangitis: Does medical treatment alter timing and selection?

Liver transplantation is a highly effective treatment for patients with adv anced primary biliary cirrhosis and primary sclerosing cholangitis, Transpl antation is indicated when the patient's survival with transplantation is b etter than without or, earlier than this, it the patient's quality of life is intolerable from intractable fatigue or pruritus, Medical therapies for chronic cholestatic liver diseases are very limited. Ursodeoxycholic acid t herapy in primary biliary cirrhosis reduces cholestasis and prolongs transp lant-free survival; no other drugs are of proven efficacy in primary biliar y cirrhosis, and none have any benefit on the disease progression of primar y sclerosing cholangitis. Aggressive endoscopic therapy may produce symptom atic and biochemical improvement in primary sclerosing cholangitis but shou ld be done without the expectation of retarding disease progression, Biliru bin is one of five criteria of the Child-Turcotte-Pugh score, which is nece ssary for the United Network for Organ Sharing listing for orthotopic liver transplantation, In addition, it is a major prognostic indicator in all th e predictive models for primary biliary cirrhosis. Bilirubin reduction with ursodeoxycholic acid therapy in primary biliary cirrhosis appears to paral lel disease severity, and prognostic models utilizing bilirubin retain thei r predictive power for survival even in treated patients. In summary, medic al therapies for chronic cholestatic liver disease have very little effect on disease progression and, subsequently, on the timing or selection for tr ansplantation, Liver transplantation is the only definitive therapy for pri mary biliary cirrhosis and primary sclerosing cholangitis. Copyright (C) 19 98 by the American Association for the Study of Liver Diseases.