Geoepidemiology of autoimmune liver diseases

Geoepidemiology of autoimmune liver diseases
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DOI:
10.1016/j.jaut.2009.12.002
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发表时间:
2010-05-01
影响因子:
12.8
通讯作者:
Invernizzi, Pietro
Invernizzi, Pietro
中科院分区:
医学1区
文献类型:
--
作者:
Invernizzi, Pietro

文献摘要

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自身免疫性肝病的特征在于胆管或肝细胞的免疫介导的损伤,因此包括胆管病如原发性胆汁性肝硬化、原发性硬化性胆管炎和免疫球蛋白G4相关胆管炎以及自身免疫性肝炎。尽管肝脏是最早被认识到的自身免疫性侵袭的部位之一,但自身免疫性肝病的病因在很大程度上仍然不清楚,其临床管理仍然困难。由于免疫学对各种慢性疾病的适用性不断增加,因此在过去几年中,关于自身免疫性肝病的基础和临床知识迅速增长。本次审查将主要集中在这些疾病的现有地理流行病学数据,但它也将处理其主要的临床特征,以及发病机制,为上述每一种疾病,连同其重叠形式。特别是,我们将讨论发展的主要潜在免疫分子机制,遗传影响,越来越多的免疫血清学诊断标志物,以及越来越有效的治疗可能性。(C)2009爱思唯尔有限公司保留所有权利。
Autoimmune liver diseases are characterized by immune mediated injury of bile ducts or hepatocytes, thus including cholangiopathies such as primary biliary cirrhosis, primary sclerosing cholangitis, and immunoglobulin G4-associated cholangitis, and autoimmune hepatitis. Although the liver was one of the earliest recognized sites of autoimmune aggression, the aetiology of autoimmune liver diseases remains largely obscure and their clinical management still difficult. Since an ever increasing applicability of immunology to a wide variety of chronic diseases, basic and clinical knowledge on autoimmune liver diseases grow rapidly in the last few years. This review will mainly focus on the available geoepidemiology data of these disorders, but it will deal also on their main clinical characteristics, as well as mechanisms of etiopathogenesis, for each of the above diseases, together with their overlap forms. In particular, we will discuss the major underlying immunomolecular mechanisms of development, the genetic influences, the growing number of immuno-serological diagnostic markers, and the increasingly effective therapeutic possibilities. (C) 2009 Elsevier Ltd. All rights reserved.