原发性胆汁性肝硬化患者自身抗体检测的临床意义  

Detection of autoimmune anticodies in patients with primary biliary cirrhosis

作　　者：王培之[1] 张黔英[1] 兰孟东[1] 郎振为[1] 

机构地区：[1]北京地坛医院病理科,北京100011

出　　处：《临床荟萃》2006年第2期103-106,共4页Clinical Focus

摘　　要：目的了解抗线粒体M2亚型抗体(AMA-M2)阳性原发性胆汁性肝硬化(PBC)患者自身抗体的种类和临床意义。方法92例AMA-M2阳性PBC患者,应用间接免疫荧光法,采用肝脏马赛克,包括猴肝和心肌、大鼠肝、胃、肾脏和人类上皮培养细胞(Hep-2)为组织基质检测血清中的自身抗体;并用免疫印迹法检测AMA亚型(M2、M4和M9)、可提取的核抗原(ENA)抗体[核糖核蛋白/史密斯抗体(nRNP/Sm)、史密斯抗体(Sm)、干燥综合征抗原A抗体(SS-A)、干燥综合征抗原B抗体(SS-B)、硬皮病70抗体(Scl-70)、Jo-1抗体(Jo-1)、着丝点B蛋白抗体(CENP B)、双链DNA抗体(dsDNA)、组蛋白抗体(histones)和抗核糖核酸P蛋白抗体(rib-P-protein)]和肝病相关自身抗体。结果92例PBC患者中有73例(79.35%)血清中存在抗核抗体(ANA);其荧光模式分别为核膜型42例(45.65%),着丝点型18例(19.57%),核浆颗粒16例(17.39%)和核点型12例(13.02%);ENA检测结果:CENP B阳性18例,SS-A阳性6例,SS-A+SS-B阳性3例,组蛋白抗体和nRNP/Sm阳性各1例;AMA分型检测中单一AMA-M2阳性62例(67.39%),AMA-M2+M4阳性26例(28.26%),AMA-M2+M4+M9阳性3例;还发现AMA-M2伴M4阳性的患者全部为疾病进展到肝硬化的阶段。结论大多数PBC患者血清中存在ANA,其荧光模式主要为核膜、着丝点、核浆颗粒和核点型;AMA-M2和M4亚型抗体为诊断PBC的重要血清免疫学标志,并且AMA-M4与疾病进程密切相关。Objective To understand the type and clinical significance of anti-autoimmune antibodies in patients with anti-mitochondria Ⅱ antibody （AMA-M2） positive primary biliary cirrhosis （PBC）. Methods Anti-nuclear antibody（ANA） was detected by indirect immunofluorescence（IIF） in 92 patients with AMA M2 positive PBC on liver mosaic including monkey liver and heart, rat liver, stomach, kidney and human epithelial （Hep-2） ceils. Subtype of AMA profile （M2, M4, M9）, antibodies against to extractable nuclear antigens（ENA） including nRNP/Sm, Sm, SS-A, SS-B,Scl-70,Jo-1 ,CENP 13, dsDNA, histones and ribosomal P-protein and anti-autoimmune antibodies associated with liver diseases were detected by immuno blotting. Results ANA was detected in 73 of 92 patients （79.35% ）. The fluorescence pattern of ANA included nuclear envelope staining 42 patients （45.65 %）, centromere antibody（ACA） in 18 patients （19.57%）,speckled 16 patients （17.39%）,and nuclear dot staining 12 patients （13.04%）. The results of ENA detection： CENP B, SS-A, SS-A/SS-B, histones and nRNP/Sm were present in 18, 6, 3, 1 and 1 patients, respectively. Alone AMA-M2,AMA-M2＋M4 and AMA-M2＋M4＋M9 were present in 62 （67.39%） ,26（28.26%） ,3 （3.26 %） patients,respectively. Conclusion ANA was expressed in most PBC patients. The distributive patterns were mainly nuclear membrane, eentromere, speckled and nuclear dot. AMA-M2 and M4 subtype are important seroimmunological markers for the diagnosis of PBC. Subtype AMA-M4 was closely related with the progressing of PBC.

关 键 词：肝硬化 抗体多样性 线粒体 自身抗体 

分 类 号：R593.2[医药卫生—内科学]