Alexandra Danningerová-Molnárová, Marian Bátovský
Primary sclerosing cholangitis (PSC) is a primary cholestatic syndrome of unknown aetiology, characterized by diffuse inflammation of the intrahepatic and/or extrahepatic bile ducts in the form of concentric obliterative fibrosis, resulting in the formation of stenosis and strictures. The disease usually leads into biliary cirrhosis and end-stage liver disease - cirrhosis. The disease and its course are changeable; it is rather a slow, prolonged illness, which often leads to death because of the associated complications. The only effective treatment for PSC is liver transplantation. The cause of PSC is unknown; it is assumed that this is a conditional autoimmune process directed against the biliary epithelial cells. A case report is presented by which we would like to point out the fact that PSC, like in primary biliary cirrhosis, has a genetic predisposition, which does not exclude the transfer of information from a relative liver donor to the recipient. Genetic factors play a major role in the development of PSC. It is known that the prevalence of PSC is significantly increased in relatives of affected individuals.