Department of Pediatrics, Interdisciplinary Laboratory of Medical Investigation, Federal University of Minas Gerais, Belo Horizonte, Minas Gerais 30130-100, Brazil.
World J Gastroenterol. 2013 Jul 28;19(28):4455-63. doi: 10.3748/wjg.v19.i28.4455.
Autoimmune hepatitis (AIH) is a rare chronic inflammatory disease of the liver, which affects a group of patients who lost their immunological tolerance to antigens of the liver. It is clinically characterized by hypergammaglobulinemia, elevated liver enzymes, presence of autoantibodies and histological changes. Although being rare in children, it represents a serious cause of chronic hepatic disease that can lead to cirrhosis and hepatic failure. Clinical findings, exclusion of more common liver disorders and the detection of antibodies antinuclear antibodies, smooth muscle antibodies and anti-LKM1 are usually enough for diagnosis on clinical practice. The pathogenic mechanisms that lead to AIH remain obscure, but some research findings suggest the participation of immunologic and genetic factors. It is not yet knew the triggering factor or factors that stimulate inflammatory response. Several mechanisms proposed partially explain the immunologic findings of AIH. The knowledge of immune factors evolved might result in better markers of prognosis and response to treatment. In this review, we aim to evaluate the findings of research about genetic and immune markers and their perspectives of application in clinical practice especially in pediatric population.
自身免疫性肝炎（AIH）是一种罕见的肝脏慢性炎症性疾病，影响一组对肝脏抗原失去免疫耐受的患者。其临床特征为高γ球蛋白血症、肝酶升高、自身抗体存在和组织学改变。虽然在儿童中罕见，但它是导致肝硬化和肝衰竭的一种严重慢性肝脏疾病的病因。在临床实践中，临床发现、排除更常见的肝脏疾病以及检测抗核抗体、平滑肌抗体和抗 LKM1 抗体通常足以进行诊断。导致 AIH 的发病机制仍不清楚，但一些研究结果表明免疫和遗传因素的参与。目前尚不清楚是什么触发因素或因素刺激了炎症反应。提出的几种机制部分解释了 AIH 的免疫发现。对免疫因素的认识的发展可能会导致更好的预后和治疗反应标志物。在这篇综述中，我们旨在评估关于遗传和免疫标志物的研究结果及其在临床实践中的应用前景，特别是在儿科人群中的应用前景。
