Autoimmune hepatitis-standard and second-line therapy

Autoimmune hepatitis (AIH) is a rare chronic inflammatory liver disease. Like other autoimmune diseases, the incidence continues to rise and, if left untreated, it has a detrimental natural course. Description of the current etiology, diagnosis and management of AIH. Summary of current national as well as international guidelines and highlighting recently published studies. In addition to autoantibody testing and liver histology, the diagnosis of AIH is based on the exclusion of other relevant liver diseases. The standard of care with prednisoloneaEuro +/- azathioprine, which has been well established for decades, significantly improves long-term survival. The therapeutic aim is the complete normalization of aminotransferases and immunoglobulin G. Budesonide, an alternative topical steroid with fewer systemic side effects, was approved for AIH therapy in non-cirrhotic patients in 2011. The therapeutic goal of complete biochemical remission is achieved in about 80% of patients and liver transplantation is rarely necessary. The majority of patients require life-long immunosuppressive therapy because of high relapse rates after discontinuation of immunosuppressants. Currently used second-line therapies are based on uncontrolled monocentric studies with single substances. Therefore, clear recommendations from international expert groups are lacking. These second-line therapies are not approved by the regulatory authorities such as the Food and Drug Administration or the European Medicines Agency.