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自身免疫性肝炎的鉴别诊断:自身抗体分型及其他。

Discrimination of autoimmune hepatitis: autoantibody typing and beyond.

机构信息

Department of Medicine, University of Vermont, Given Building, Burlington, VT 05405-0068, USA.

出版信息

J Gastroenterol. 2011 Jan;46 Suppl 1:39-41. doi: 10.1007/s00535-010-0324-3. Epub 2010 Sep 23.

Abstract

For over 20 years autoimmune hepatitis has been classified into types 1 and 2, an autoantibody-based classification that has stood the test of time. In type 1 autoimmune hepatitis, the main circulating autoantibodies, although not specific for the disease, are antinuclear antibodies, smooth-muscle antibodies, and anti F-actin antibodies. Soluble liver antigen antibodies have been found in approximately 10-30% of adult patients with type 1 disease, and are more common in children with type 1 and type 2 autoimmune hepatitis. Atypical p-antineutrophil cytoplasmic autoantibodies frequently occur in type 1 autoimmune hepatitis but are non-specific. Antimitochondrial antibodies, although more specific and sensitive for primary biliary cirrhosis, are seen in type 1 autoimmune hepatitis, generally in conjunction with antinuclear and/or smooth-muscle antibodies. In type 2 autoimmune hepatitis, a disease which occurs predominantly in girls and young women, anti-liver-kidney microsomal-1 antibodies and anti-cytosol-1 antibodies are the major circulating autoantibodies. There also are patients with variant forms of autoimmune hepatitis who have clinical and serologic findings of autoimmune hepatitis in addition to features of primary biliary cirrhosis or primary sclerosing cholangitis. The taxonomy and definitions of these variants, often referred to as overlap syndromes, are controversial. IgG4-associated cholangitis is recognized as an entity distinct from autoimmune hepatitis. Whether or not IgG4-associated autoimmune hepatitis is a distinct entity awaits a more extensive description of clinical and immuno-histological features.

摘要

20 多年来,自身免疫性肝炎已分为 1 型和 2 型,这是一种基于自身抗体的分类方法,经受住了时间的考验。在 1 型自身免疫性肝炎中,主要循环自身抗体虽然不是该疾病的特异性抗体,但包括抗核抗体、平滑肌抗体和抗 F-肌动蛋白抗体。在约 10-30%的 1 型疾病成年患者中发现可溶性肝抗原抗体,在 1 型和 2 型自身免疫性肝炎的儿童中更为常见。非典型 p-中性粒细胞胞浆抗体常发生于 1 型自身免疫性肝炎,但无特异性。抗线粒体抗体虽然对原发性胆汁性肝硬化更特异和敏感,但也见于 1 型自身免疫性肝炎,通常与抗核抗体和/或平滑肌抗体同时出现。在以女孩和年轻女性为主的 2 型自身免疫性肝炎中,抗肝-肾微粒体-1 抗体和抗胞浆-1 抗体是主要的循环自身抗体。还有一些变异型自身免疫性肝炎患者,除原发性胆汁性肝硬化或原发性硬化性胆管炎的特征外,还具有自身免疫性肝炎的临床和血清学表现。这些变异型(通常称为重叠综合征)的分类和定义存在争议。IgG4 相关胆管炎被认为是一种与自身免疫性肝炎不同的实体。IgG4 相关自身免疫性肝炎是否是一种独特的实体,有待更广泛地描述其临床和免疫组织学特征。

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