20例原发性胆汁性肝硬化自身抗体和免疫功能分析

Autoantibodies and Immunologic Function in Primary Biliary Cirrhosis: 20 Cases Analysis

背景:一般认为血清抗线粒体抗体M2亚型(AMA-M2)阳性是诊断原发性胆汁性肝硬化(PBC)的必备标准,但仍存在一定比例的AMA-M2阴性PBC患者。因此,了解PBC患者的自身抗体谱和免疫功能有助于PBC的诊断。目的:分析PBC患者的自身抗体和免疫功能,探讨其对PBC的诊断价值。方法:连续纳入确诊的PBC患者20例,以免疫印迹法检测肝特异性自身抗体,以间接免疫荧光法检测肝非特异性自身抗体,以免疫散射比浊法检测免疫球蛋白和补体;13例患者行肝穿刺活检组织病理学检查。结果:本组PBC患者女性多于男性(17:3),年龄39～75岁,平均57.2岁±2.1岁。肝特异性自身抗体中AMA-M2阳性最为多见(40%),其次为核点蛋白SP100抗体(15%)、肝肾微粒体1型(LKM-1)抗体(15%)、GP210抗体(10%)和肝细胞溶质抗原Ⅰ型(LC-1)抗体(10%);肝非特异性自身抗体以抗核抗体(ANA)检出率最高(70%);IgA、IgM和IgG升高者均占50%。结论:自身抗体,尤其是血清AMA-M2阳性是诊断PBC的重要依据之一,但多种蛋白在PBC病程中均有表达,且临床上有相当比例的AMA-M2阴性PBC患者。其他肝特异性自身抗体,如SP100抗体、GP210抗体等以及临床特点和肝穿刺活检对AMA-M2阴性PBC的诊断有重要价值。

Background： Serum antimitochondrial antibody-M2 （AMA-M2） is considered to be a prerequisite for the diagnosis of primary biliary cirrhosis （PBC）, however, there are a portion of AMA-M2-negative PBC patients. Thus it is very important to understand the autoantibodies spectrum and immunologic function for the diagnosis of PBC. Aims： To investigate the autoantibodies, immunologic function and their diagnostic value in PBC. Methods： Twenty cases of consecutive PBC patients were enrolled in the study. Liver-specific autoantibodies were detected by immunoblotting, non-liver-specific autoantibodies by indirect immunofluorescence, and immunoglobulin and complement by nephelometry. Liver biopsies were processed for histopathology in 13 PBC patients, Results： Among the patients enrolled in this study, 3 were males and 17 females, the age ranged from 39 to 75 years with the mean age 57.2 years±2.1 years. The positivity rates of liverspecific autoantibodies were as follows： AMA-M2 40%, nuclear dot-associated protein SP100 antibody 15%, liver-kidney microsomes type 1 （LKM-1） antibody 15%, GP210 antibody 10%, and liver cytosolic antigen type 1 （LC-1）antibody 10%. The positivity rate of non-liver-specific autoantibody antinuclear antibody （ANA） was 70%. Increased IgA, IgM and IgG were found in 10 patients, respectively. Conclusions： Autoantibodies are present in PBC patients, and serum AMA-M2 is one of the most important indexes in the diagnosis of PBC, however, other proteins also express in the course of disease. There are a portion of AMA-M2-negative PBC patients in the clinics, Therefore, other liver-specific autoantibodies such as SP100 antibody, GP210 antibody are important in the diagnosis of AMA-M2-negative PBC, and the clinical features as well as liver biopsy are also invaluable.