Biologie des maladies cholestatiques chroniques de l'adulte

Cholestasis is defined as a defect in bile acid transport from the liver to the intestine. Biochemical tests of cholestasis reflect, on one hand, hepatocellular or cholangiocellular cell damage induced by bile acids (liver enzymes) and, on the other hand, functional consequences of altered bile secretion (serum bilirubin and bile acids). Liver biopsy still has a role in the diagnosis of chronic cholestasis. Main chronic cholestatic diseases in adults are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Prognostic factors have been identified. Liver stiffness measurement by vibration-controlled transient elastography is a very promising tool in the management of both PBC and PSC. Medical therapy is based on ursodeoxycholic acid. However, a number of novel anti-cholestatic agents, especially nuclear receptor agonists including obeticholic acid and fibrates, have undergone pre-clinical and clinical evaluation and have shown promising results. Better times for these patients seem likely in the near future.