Abstract
Background: Autoimmune hepatitis (AIH) is a hepatocyte injury characterized by a dis-regulated immune system, inflammation of the liver with interface hepatitis, serum antibodies and elevated gamma-globulins. Currently, it exists 2 mainly types on autoimmune hepatitis, based on the presentation of their autoantibodies: type 1 and 2. Type 1 is the one of interest in this review. Autoimmune hepatitis type 1 is an indolent inflammatory disease mediated by abnormal autoimmune reaction, commonly seeing in women (4:1), with no preference of age, however typically present in 4th and 6th decade’s individuals, and a good response to immunosuppressive therapy. Autoimmune hepatitis is known to present not specific symptoms which can go from inexistent to fulminant or exacerbate presentation. The incidence of autoimmune hepatitis in western country is 1/5000 - 1/10,000. Although the detection rate of AIH increasing, especially in China, autoimmune hepatitis diagnosis and treatment remain challenging. Most AIH case do not present pathognomonic biomarkers, however the diagnostic has to considerate other features, which had been codified into validated diagnosis scoring systems. Regarding the treatment, based on prior literature autoimmune hepatitis is mostly sensitive to prednisone or azathioprine, however the use of other immunosuppressive agent or event their combination had offer distinct advantages. Autoimmune hepatitis presents a great life threatening disorder especially because of the delay in the early stage on the disease, the not specific presentations, and the large overlap syndrome associate with it. A deep knowledge in its identification, specific diagnostic criteria, and its therapeutic strategy is much needed. Conclusion: Autoimmune hepatitis is a systemic disease that is difficult to recognize because of its variable clinical presentation and histological features that are not strictly specific. Several new autoantibodies as well as recently simplified diagnostic criteria may allow the primary care physician to progress in the diagnostic process. This is all the more important as undiagnosed and therefore untreated autoimmune hepatitis has a poor prognosis and immunosuppressive treatment leads to remission in a large majority of cases.
Highlights
Autoimmune hepatitis firstly described by Waldenström and Kunkel in the 1950s, inherited a variety of nomenclature, including chronic active hepatitis, cirrhosis of young women and plasma cell hepatitis [1]
Autoimmune hepatitis type 1 is an indolent inflammatory disease mediated by abnormal autoimmune reaction, commonly seeing in women (4:1), with no preference of age, typically present in 4th and 6th decade’s individuals, and a good response to immunosuppressive therapy
Autoimmune hepatitis (AIH) is considered as a disorder with atypical clinical and histological features, based on the literature Caucasian woman has no exclusivity for autoimmune hepatitis, she has long been considered the typical terrain
Summary
Autoimmune hepatitis firstly described by Waldenström and Kunkel in the 1950s, inherited a variety of nomenclature, including chronic active hepatitis, cirrhosis of young women and plasma cell hepatitis [1]. An immune response to an external pathogen, drug or toxin trigger could erroneously target structurally similar internal components (mimicry, molecular). In case of dysfunction of regulatory T cells, which inter alia prevent autoimmunity, the mechanism of destruction of hepatocytes may persist The latter is mediated in AIH by cytotoxic T lymphocytes (CD8 + T), cytokines secreted by Th1 lymphocytes, monocytes, macrophages, NK lymphocytes and complement activation [6] [7]. The excellent specificity of these simplified criteria has been confirmed in an even more recent study; this method is wildly used in clinical practice, its sensitivity still remain lower, so it should always make use of the original IAIHG scoring method [11]
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