Primary biliary cholangitis ( PBC ) and primary sclerosing cholangitis ( PSC ) are the most frequent chronic cholestatic liver diseases and serve as model diseases to discuss the management of cholestasis in 2017 in the lecture that is summarized in this report. PBC and PSC are characterized by inflammation and fibrosis of small intrahepatic ( PBC ) or larger intra- and/or extrahepatic ( PSC ) bile ducts. Bile duct damage leads to cholestasis and can progress to liver fibrosis and even cirrhosis. Various genetic, environmental and endogenous factors may contribute to the development of chronic cholestatic liver diseases, but the exact pathogenesis of PBC and PSC has not been clarified. Ursodeoxycholic acid ( UDCA ) is the standard treatment of PBC and is used also for other cholestatic conditions including PSC , and it exerts anticholestatic effects at adequate doses. Novel anticholestatic therapeutic options for patients not adequately responding to UDCA are under development or have, like obeticholic acid, already been proven to have efficacy when combined with UDCA in the treatment of PBC . The future role of immunomodulating/immunosuppressive drug regimens must be critically reviewed.
K E Y W O R D S
cholestasis , farnesoid X receptor , nuclear receptor agonists , peroxisome proliferator-activated
receptor α , ursodeoxycholic acid