Autoimmune liver diseases

Autoimmune liver diseases in children and adolescents include autoimmune hepatitis (AIH), autoimmune sclerosing cholangitis (ASC), primary sclerosing cholangitis (PSC), overlap syndrome and de novo autoimmune hepatitis. The clinical symptoms are mostly uncharacteristic. Apart from elevated aminotransferase concentrations, laboratory findings in serum include hypergammaglobulinemia, increased immunoglobulin G (IgG) levels and the presence of specific autoantibodies. In addition, histological investigation of liver tissue is always mandatory. Due to the frequently unremarkable clinical course, a considerable number of patients already show severe liver fibrosis or beginning liver cirrhosis at the time of diagnosis. Early diagnosis is very important because immunosuppressive treatment initiated immediately after diagnosis can prevent severe liver disease and rapid progression. The standard immunosuppressive treatment is based on the combination of prednisolone and azathioprine. In cases without remission cyclosporine A or mycophenolate mofetil can be administered. The treatment has to be continued over years and relapses are common. Treatment can be successfully completed in only 20 % of treated subjects. In 8-10 % of patients liver transplantation is necessary within 15 years after diagnosis. Follow-up examinations on a regular basis are essential.