Autoimmune Lebererkrankungen

Abstract

Autoimmune liver diseases in children and adolescents include autoimmune hepatitis, autoimmune cholangitis, and sclerosing cholangitis. The etiology is widely unknown. Besides mostly uncharacteristic clinical symptoms diagnostic criteria are elevated aminotransferases, hypergammaglobulinemia, increased IgG level, and the presence of specific autoantibodies. Additionally histological investigation of liver tissue is mandatory. A considerable number of patients show severe liver fibrosis or beginning liver cirrhosis at diagnosis. Early diagnosis is most important, because immediate immunosuppressive treatment may prevent rapid progression of liver disease. Immunosuppressive treatment is still based on the combination of prednisolone and azathioprine. In cases without remission cyclosporin or mycophenolate mofetil can be administered. In most cases long-term treatment is necessary, as relapses are common. Only in 20% of treated subjects may therapy be successfully stopped. In 8–10% of patients liver transplantation may be indicated 15 years after diagnosis.