中文摘要：

目的分析中国人原发性胆汁性肝硬化（PBC）的实验室指标特征,为PBC实验室诊断提供参考。方法对10余年来保存的361例特异性AMA-M2抗体阳性PBC患者的血清标本,检测分析自身抗体、血清酶谱、免疫球蛋白等指标,并用免疫印迹法分析M2靶抗原分布情况。结果 361例血清标本中最常见的生化指标异常为ALP和GGT,异常率分别为84.5%和78.9%,有71.7%的标本IgM异常增高。14.7%（53/361）ALT、ALP和GGT等生化指标全部正常。AMA和ANA阳性率分别为84.5%和76.7%,在ANA阳性标本中,核膜型与着丝点型为最常见的荧光图形。M2型抗体的三个主要靶抗原PDC-E2、BCOADC-E2和OGDC-E2在本研究患者中的阳性率分别为85.3%、69.5%和24.4%。结论 AMA-M2抗体阳性的中国人PBC,仍以血清IgM、ALP、GGT水平升高和AMA阳性常见,但14.7%PBC患者初诊时ALT、ALP和GGT等生化指标正常。三个主要M2型靶抗原分布不同于白种人报道。

英文摘要：

Objective To study the laboratory features of primary biliary cirrhosis （PBC） in Chinese so as to provide the reference for PBC laboratory diagnoisis.Methods Three hundred and sisty-one stored AMA M2 autoantibodies positive serum samples which have been kept for 10 years were used to analyze the autoantibodies,biochemical abnormalities and immunoglobulins retrospectively.Results The most frequently biochemical abnormalities were alkaline phosphatase （ALP） （84.5%） and gamma glutamyl transpeptidase （GGT） （78.9%）.The levels of IgM increased abnormally in 259 patients （71.7%）.Biochemical indicators were normal in 53 AMA-M2 positive patients.Positive rates of AMA and ANA were 84.5% and 76.7% respectively.The main fluorescent patterns of ANA were nuclear membrane and centromere patterns.The positive rate of antibodies to the major autoantigens PDC-E2,BCOADC-E2 and OGDC-E2 were 85.3%,69.5% and 24.4% respectively in Chinese patients.Conclusion Together with a positive AMA-M2,elevated serum IgM,ALP,GGT levels and AMA positive were most freguently found in PBC patients.14.7% patients had normal biochemical indicators including ALT,ALP and GGT at the time of first diagnosis.Distributions of three major AMA-M2 autoantigens in Chinese patients were different from those in caucasian.