Autor: |
van Gerven NM; Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands., de Boer YS; Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands., Mulder CJ; Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands., van Nieuwkerk CM; Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands., Bouma G; Nicole MF van Gerven, Ynto S de Boer, Chris JJ Mulder, Carin MJ van Nieuwkerk, Gerd Bouma, Department of Gastroenterology and Hepatology, Vu University Medical Centre, 1081 HV, Amsterdam, The Netherlands. |
Abstrakt: |
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. |