Splošna bolnišnica Izola, Izola, Slovenija
Klinični oddelek za gastroenterologijo, hepatologijo in nutricionistiko, Pediatrična klinika, Univerzitetni klinični center Ljubljana, Ljubljana, Slovenija
Autoimmune liver diseases are chronic diseases of the liver caused by an autoimmune process in the liver – autoimmune hepatitis, in the biliary tract – autoimmune sclerosing cholangitis, in both the liver and biliary tract – overlap syndrome, de novo autoimmune hepatitis following liver transplantation and Coombs’ positive giant cell hepatitis. Standard treatment is with a combination of corticosteroids and azathioprine, which results in remission in 80 % of children. The non-responders are treated with more potent immunosuppressive drugs. If autoimmune liver disease progresses to cirrhosis of the liver despite more intensive treatment, the child requires liver transplantation.
Key words: autoimmune hepatitis, autoimmune sclerosing cholangitis, children, treatment.