자가면역 간질환 = Metabolic & Immunologic Disorders and Liver : Autoimmune liver diseases

다국어 초록 (Multilingual Abstract)

Autoimmune liver disease is an undefined immune mediated attack to the hepatocyte, small intrahepatic bile ducts, and the entire biliary system leading to chronic inflammation and fibrosis. It includes autoimmune hepatitis (AIH), primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), IgG4-associated cholangitis (IAC), and variant forms such as overlap syndrome. PBC, PSC and IAC are cholestatic diseases showing different clinical features, diverse serological profiles, and different therapeutic regimens. AIH is characterized by circulating autoantibodies, elevated immunoglobulins, and dramatic response to immunosuppression. Despite of distinction of two main disorders, some patients showed an overlap disease with mixed features of both disorders. To prevent the progression to the end stage liver disease, early and precise diagnosis and initiation of therapeutic measures are essential. Liver transplantation is indicated for the treatment refractory advanced cirrhosis. Further studies on the autoimmune liver diseases in Korea are warranted.