Autoimmune Hepatitis: Clinical Review with Insights into the Purinergic Mechanism of Disease.
Autoimmune Hepatitis: Clinical Review with Insights into the Purinergic Mechanism of Disease.
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DOI：
10.14218/jcth.2013.00015
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发表时间：
2013-12
影响因子：
3.6
通讯作者：
Robson SC
中科院分区：
文献类型：
--
作者：
Kapila N;Higa JT;Longhi MS;Robson SC
自身免疫性肝炎（AIH）是一种重要的疾病，主要导致炎症性肝病的遗传易感妇女。临床病理特征为全身性炎症和肝功能障碍相关症状，以及血清转氨酶升高、IgG升高、自身抗体和肝活检界面肝炎。AIH通常会导致肝损伤，这是慢性肝炎和肝硬化的结果。然而，在极少数情况下，患者可能会出现暴发性肝功能衰竭。早期诊断在所有情况下都很重要，因为这种疾病对免疫抑制治疗方案具有高度反应性。如果不加以治疗，这种疾病的发病率和死亡率都很高。在这里，我们提供了一个关于AIH的知识现状的概述，并总结了成人这种严重疾病的治疗方案。我们还讨论了发病机制的疾病作为一个可能的后果，自身免疫和肝耐受性的崩溃。我们专注于调节性T细胞损伤的CD39外核苷酸酶的表达和改变嘌呤能信号的变化的结果。进一步了解肝脏耐受性可能有助于开发特异性和耐受性良好的AIH治疗方法。
Autoimmune hepatitis (AIH) is an important disorder that predominantly results in inflammatory liver disease in genetically predisposed women. The clinicopathological picture is characterized by symptoms associated with both systemic inflammation and hepatic dysfunction, and with increased serum aminotransferases, elevated IgG, autoantibodies, and interface hepatitis on liver biopsy. AIH usually results in liver injury as a consequence of chronic hepatitis and cirrhosis. However, rarely, patients may present with fulminant liver failure. Early diagnosis is important in all instances because the disease can be highly responsive to immunosuppressive therapeutic options. Left untreated, the disease is associated with high morbidity and mortality. Here we provide an overview of the current state of knowledge on AIH and summarize the treatment options for this serious condition in adults. We also discuss the pathogenesis of the disease as a possible consequence of autoimmunity and the breakdown of hepatic tolerance. We focus on regulatory T cell impairments as a consequence of changes in CD39 ectonucleotidase expression and altered purinergic signaling. Further understanding of hepatic tolerance may aid in the development of specific and well-tolerated therapies for AIH.