Autoimmune hepatitis – Update 2015

Autoimmune hepatitis (AIH) was first described in 1951 [1] as a chronic hepatitis of young women with hypergammaglobulinemia in the absence of cirrhosis, which responds well to adrenocorticotrophic therapy (ACTH). Shortly thereafter this syndrome was described and characterized in the USA. In 1956 the association with anti-nuclear antibodies (ANA) was discovered and the term ‘‘lupoid hepatitis’’ was created [2]. AIH and systemic lupus erythematosus (SLE) are distinct autoimmune disorders. However, they may occur together in a given patient. Between 1960 and 1980 several prospective trials were published demonstrating the benefits of corticosteroids alone or in combination with azathioprine in severe cases of AIH. AIH became the first liver disease in which medical therapy improved survival [3]. The advent of immunofluorescence, thereafter radio, as well as enzyme-linked immunosorbent (EIA) assay technology, in addition to molecular cloning techniques allowed a molecular identification and characterization of the hepatocellular autoantigens involved in AIH (Table 1). Characterization of the humoral and cellular immune system in patients and several animal models significantly improved our knowledge of this still poorly understood autoimmune liver disease (Fig. 1). Immunosuppression and liver transplantation are our therapeutic weapons. While corticosteroids alone or in combination with azathioprine are effective and prolong survival, treatment failures to this standard of care (SOC) are still a challenge. This review on the occasion of the 30 year anniversary of the Journal of Hepatology and the 50 year anniversary of the European Association for the Study