Auto immune hepatitis

Abstract

To provide an update of the latest trends in epidemiology, clinical course, diagnostics, complications and treatment of auto immune hepatitis (AIH). A search of the MEDLINE database was performed using the search terms: “auto immune hepatitis”, “clinical presentation”, “symptoms”, “signs”, “diagnosis”, “auto antibodies”, “laboratory values”, “serology”, “histopathology”, “histology”, “genetics”, “HLA genes”, “non-HLA genes”, “environment”, “epidemiology”, “prevalence”, “incidence”, “demographics”, “complications”, “HCC”, “PBC”, “PSC”, “corticosteroid”, “therapy”, “treatment”, “alternative treatment”. English-language full-text articles and abstracts were considered. Articles included reviews, meta-analysis, prospective retrospective studies. No publication date restrictions were applied. AIH is an immune meditated progressive inflammatory liver disease that predominantly affects middle-aged females but may affect people of all ages. The clinical spectrum of AIH is wide, ranging from absent or mild symptoms to fulminant hepatic failure. The aetiology of AIH is still unknown, but is believed to occur as the consequence of an aberrant immune response towards an un-known trigger in a genetically susceptible host. In the absence of a gold standard, diagnosis is based on the combination of clinical, biochemical and histopathological criteria. Immunosuppressive treatment has been the cornerstone of treatment since the earliest description of the disease in 1950 by Waldenström. Such treatment is often successful at inducing remission and generally leads to normal life expectancy. Nevertheless, there remain significant areas of unmet aetiological a clinical needs including fundamental insight in disease pathogenesis, optimal therapy, duration of treatment and treatment alternatives in those patients unresponsive to standard treatment regimens.

Keywords: Auto immune hepatitis, Diagnosis, Liver, Epidemiology, Treatment

Core tip: Autoimmune hepatitis (AIH) is a chronic inflammatory liver disorder of unknown aetiology, which can lead to hepatic failure and premature death when untreated. In AIH there is no existence of a pathognomonic feature and therefore the diagnosis rests on a combination of immunological, biochemical, and histological features together with exclusion of other liver diseases. Due to large heterogeneity of the disease, AIH might be unrecognised. Immunosuppressive treatment has been the cornerstone of treatment. Such treatment is often successful at inducing remission. For most patients life long treatment is indicated. In patients in whom all treatments fail, liver transplantation remains a final option.