Description
Several primary liver diseases are considered to have an autoimmune basis. There is a clinical, immunological and histopathological spectrum and overlap syndromes exist but there are three classical examples of autoimmune liver disease autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis. The histopathologist is able to define the principal processes present in liver biopsy specimens (ie. hepatitic or cholestatic; bile duct injury or interface hepatitis) and can identify the stage of the disease. However a definitive diagnosis can only be made following correlation with the clinical, biochemical and immunological findings. This review describes the classical forms of autoimmune liver disease and briefly considers variant and overlap syndromes.
