AMA-M2阳性的原发性胆汁性肝硬化患者免疫学特点分析

Immunological characteristics of primary biliary cirrhosis patients with AMA-M2 positive

目的分析抗线粒体M2亚型抗体阳性(AMA-M2)的原发性胆汁性肝硬化(PBC)患者免疫学等实验室诊断特点,推进临床PBC的早期诊断和治疗。方法对400例AMA-M2阳性的PBC患者采用间接免疫荧光法检测血清ANA、AMA、SMA、LKM和PCA等5种自身抗体。采用免疫印迹法检测患者血清7种主要肝抗原:Sp100、PML、gp210、LKM-1、LC-1、SLA/LP和Ro-52。应用全自动生化分析仪检测患者ALP、γ-GT等生化指标,全自动血细胞分析仪和凝血分析仪检测患者WBC、RBC、HGB、PLT和PT等血液学指标。结果患者自身抗体检测:ANA荧光模型主要包括核膜型、多核点型、着丝点型和胞浆颗粒型,阳性率分别为70%、52%、43%、3%。荧光模型组合方式包括:多核点型+核膜型、着丝点型+核膜型、着丝点型、多核点型、胞浆颗粒型以及仅AMA阳性者,阳性率分别为42%、28%、15%、10%、3%、2%,荧光滴度均≥1∶320。SMA、PCA、LKM阳性率均低于5%。患者主要肝抗原为Ro-52、gp210、PML和Sp100,阳性率分别为53%、44%、43%、24%,荧光滴度≥1∶320,而LKM-1、LC-1、SLA/LP阳性率均低于5%。PML、Sp100及Sp100+PML与gp210的阳性一致率分别为29%、11%、7%。临床血液检测结果显示有41%的患者WBC和RBC降低,53%的患者HGB降低,38%的患者PLT降低,而有42%的患者PT延长。生化检测结果显示有96%和93%的患者ALP和γ-GT升高。结论 AMA-M2阳性的PBC患者具有高滴度、特征性自身抗体荧光模型及自身肝抗原,结合临床血液及生化检测结果将为临床诊断提供重要帮助。

Objective To study the immunological characteristics of primary biliary cirrhosis patients with anti-mitochondrial antibody M2 subtype （AMA-M2） positive,for promoting early diagnosis and treatment of PBC.Methods Five kinds of autoantibodies including ANA,AMA,SMA,LKM and PCA in serum from 400 PBC patients with AMA-M2 positive were analyzed by indirect immunofluorescence assay and seven kinds of liver antigens including Sp100,PML,gp210,LKM-1,LC-1,SLA/LP and Ro-52 were tested by immunoblotting test.Biochemical indicators of ALP and γ-GT were assaied by automatic biochemical analyzer.Clinical hematology indicators of WBC,RBC,HGB,PLT and PT were assaied by automatic blood cell analyzer and automatic coagulation analyzer.Results The patients＇ main florescence patterns of ANA were nuclear envelope,multiple nuclear dots,centromere pattern and cytoplasm particles pattern,the positive rates were 70％,52％,43％ and 3％.Fluorescent pattern combination models included multiple nuclear dots plus nuclear envelope,centromere pattern plus nuclear envelope,centromere pattern,multiple nuclear dots,cytoplasm particles pattern and only AMA positive pattern.The positive rate of those models were 42％,28％,15％,10％,3％,2％,and the titers were all beyond 1：320.The positive rate of SMA,PCA,LKM were all less than 5％.The main liver antigens of patiens were Ro-52,gp210,PML,Sp100 and the positive rate were 53 ％,44％,43％ and 24％ respectively,and the titers were all beyond 1：320.The positive rate of other liver antigens including LKM-1,LC-1,SLA/LP were all less than 5％.Positive concordance rate of PML,Sp100 and Sp100 combined with PML to gp210 were 29％,11％ and 7％.The detection of clinical hematology indicators show that in41％ patients WBC and RBC levels reduced abnormally,in 53％ patients and 38％ patients the HGB and PLT levels reduced abnormally,while in 42％ patients PT levels prolonged abnormally.The detection of biochemical indicators showed that in 96％ and 93％ patients ALP and γ-GT levels elevated abnormally.Conclusions PBC patients with AMA-M2 positive characterized as high titers-special autoantibodies florescence patterns and liver antigens,together with abnormal biochemical indicators and clinical hematology indicators would be beneficial to diagnosis of PBC.