JLE

Hépato-Gastro & Oncologie Digestive

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Primary biliary cirrhosis Volume 20, issue 6, Juin 2013

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Authors
Université Paris 7 Diderot, Faculté de Médecine Xavier-Bichat, 16 rue Henri-Huchard 75018 Paris, France, Hôpital Saint-Antoine, service d’hépatologie, F-75012 Paris, France ; UPMC Université Paris 6, UMR_S 938, F-75012 Paris, France

Primary biliary cirrhosis is an autoimmune liver disease affecting mostly women. It is thought that the cause is a combination of environmental triggers and of a genetic predisposition. The diagnosis rests on two of the three following criteria: 1) clinical and/or biochemical signs of cholestasis (in particular increase in serum alkaline phosphatase activity) persisting more than 6 months; 2) presence of M2 antimitochondrial antibodies; 3) signs of chronic non-suppurative cholangitis at liver biopsy. The first line treatment is ursodeoxycholic acid (UDCA) at the dose of 13-15 mg/kg per day. UDCA significantly prolongs survival without transplantation in patients treated early. However, in about one-third of cases, particularly when true cirrhosis has developed with marked jaundice or ascites, liver transplantation is indicated. Recurrence after transplantation is observed in 20-25% of cases. Research should focus on on a better identification of factors triggering the disease, and on those influencing progression and treatment response.