123例原发性胆汁性肝硬化临床表现与自身抗体谱

Clinical manifestation and autoantibody profile in 123 patients with primary biliary cirrhosis

目的:探讨123例原发性胆汁性肝硬化患者的临床表现与自身抗体谱的关系。方法:选取2008年至2010年解放军三零二医院收治的123例原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者进行回顾性分析,按照临床及病理分期分为肝硬化期70例和非肝硬化期53例,采用间接免疫荧光法检测抗核抗体(anti-nuclear anti-bodies,ANA)和抗线粒体抗体(anti-mitochondrial antibodies,AMA),采用免疫印记法(EUROLINE)检测抗线粒体抗体M2亚型(anti-mitochondrial antibodies-M2,AMA-M2)、抗早幼粒细胞白血病蛋白抗体(anti-promyelocytic leukemia,anti-PML)、抗核点型靶抗原蛋白100 000(anti-sp100 antibodies,anti-sp100)、抗核孔复合物210 000跨膜糖蛋白(an-ti-gp210 antibodies,anti-gp210)、抗重组M2融合蛋白抗体(anti-BCOADC-E2 PDC-E2 OGDC-E2 antibodies,anti-BPO)、抗52 000核颗粒蛋白(anti-Ro-52 antibody,anti-52KD)。结果:ANA、AMA、AMA-M2、anti-PML、anti-sp100和anti-52KD阳性率,肝硬化期分别49%、47%、51%、54%、31%和49%,非肝硬化期分别为38%、37%、51%、60%、30%和51%,两组间比较差异均无统计学意义(P均>0.05);anti-gp210阳性率:肝硬化期27%,非肝硬化期19%,两组间比较差异有统计学意义(P<0.01);anti-gp210阳性的PBC患者较anti-gp210阴性的PBC患者白蛋白和胆碱酯酶偏低,转氨酶、胆红素、碱性磷酸酶和Mayo评分偏高,发展为肝衰竭概率大。结论:AMA有助于疾病的早期诊断,ANA阳性有助于AMA阴性患者的诊断;anti-gp210出现在PBC病程后期,anti-gp210阳性的PBC患者有更严重的胆汁淤积和肝功能损害。

Objective：To investigate the autoantibody profile and its clinical implication in the patients with primary biliary cirrhosis. Methods： During the period of 2008 to 2010,123 patients with primary biliary cirrhosis （PBC） in our hospital were enrolled in this study, of whom, 70 patients were with cir-rhosis and 53 without cirrhosis, The autoantibody profile was tested for each patient by using immunoblot-ting and indirect immunofluorescence. Results： Of the 123 PBC patients with liver cirrhosis, 49% were positive with serum ANA positive; 47%, 51%, 54%, 31% and 49% were positive with serum anti-nu- clear antibodies （ANA）, anti-mitochondrial antibodies-M2 （AMA-M2）, anti-promyelocytic leukemia （ anti-PML）, anti-spl00 antibodies （ anti-spl00）, anti-Ro-52 antibody （anti-52KD） contrast, of the PBC patients without liver cirrhosis, only 38%, 37%, 51%, 60% respectively. By , 30% and 51% were positive with serum ANA, AMA, AMA-M2, anti-PML, anti-spl00 and anti-52KD, respectively. There was the statistical difference between the two groups. In addition, it was also found that the anti-gp210 antibody positive group had a higher Mayo risk score,lower serum albumin and severe cholestasis and impaired liver function when compared with anti-gp210 antibody negative patients. Conclusion： Our data indicate that serum AMA is helpful for early diagnosis of PBC, and in particular, serum ANA posi-tivity can help make a diagnosis for the AMA-negative patients. These indicate that anti-gp210 antibodies appear in the late course of PBC. Anti-gp210 positive PBC patients have more severe cholestasis and liver dysfunction.