45

The introduction of ursodeoxycholic acid therapy has improved the natural history of primary biliary cholangitis. Patients treated at an early stage and who have a biochemical response have a normal life expectancy. Over the years, primary biliary cholangitis has acquired a reputation within the hepato-gastroenterological community as a rather indolent disease, even though 40% of patients are non-responsive to treatment and the rate of liver transplantation in this indication has not changed for 10 years. Several scores are used to measure the risk of mortality in patients treated with ursodeoxycholic acid (Globe score, UK-PBC score). Nearly 12% of mortality is premature and 80% of deaths occur before reaching life expectancy at birth. Liver transplantation for primary biliary cholangitis appears to be stable over time with an average of between 30 and 40 patients transplanted per year in France and 200 in Europe. The indications for transplantation are heterogeneous: jaundice, refractory pruritus, complications of cirrhosis, and hepatocellular carcinoma.