自身免疫肝病患者抗中性粒细胞胞浆抗体的检测及临床意义

探讨抗中性粒细胞胞浆抗体(anti-neutrophil cytoplastic antibodies,ANCA)对自身免疫肝病的临床意义。应用间接免疫荧光法和斑点法检测149例自身免疫性肝病患者自身免疫性肝炎(autoimmune hepatitis,AIH)患者57例,原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者42例,不明原因肝损患者50例]的ANCA和抗可提取性核抗原抗体(extract-able nuclear antigen,ENA),进而用ELISA法分析60例ANCA阳性的自身免疫肝病患者的ANCA抗原谱。以200例健康献血员为正常对照。结果ANCA在AIH、PBC、不明原因肝损中的阳性率分别为81%、40%、30%,其中非典型性pANCA的阳性率依次为70%、40%、28%。AIH组与PBC组及AIH组与不明原因肝损组间非典型性pANCA的阳性率有显著性差异(P<0.01),但PBC组与不明原因肝损组间差异不显著(P>0.05)。各疾病组ANCA抗原谱如下:AIH组中,乳铁蛋白3%阳性,MPO 11%阳性,组织蛋白酶G和BPI的阳性率分别为11%、17%;PBC组中,弹性蛋白酶和组织蛋白酶G阳性率均为5%,BPI的阳性率为16%;不明原因肝损组中,BPI阳性率为17%。大多数自身免疫性肝病患者非典型性pANCA为阳性(28%~70%),且伴有特征性自身抗体。注重非典型性pANCA的检测对明确诊断自身免疫肝病及其分类有很大的帮助。

Detection of anti-neutrophil cytoplastic antibodies in patients with autoimmune liver diseases and its clinical significance

To explore the clinical significance of anti-neutrophil cytoplastic antibodies(ANCA) in patients with autoimmune liver disease,ANCA and antibodies against extractable nuclear antigen(ENA) in 149 patients with autoimmune liver diseases including 57 case of autoimmune hepatitis(AIH),42 cases of primary biliary cirrhosis(PBS) and 50 cases with uncertain causes of liver damage were detected by indirect immunofluorescence and dot blotting assay.Then,the antigenic spectrum of ANCA in 60 cases of ANCA-positive patients with autoimmune liver diseases was determined by ELISA assay.Meanwhile,200 healthy individuals were employed as normal controls.It was demonstrated that the positive rates of detection of ANCA in sera of patients with AIH,PBC and liver damages with uncertain causes were 81%,40% and 30% respectively,and those of atypical perinuclear anti-neutrophil cytoplastic antibodies(pANCA) in the corresponding cases of patients were 70%,40% and 28% respectively.Significant differences in the positive rates of pANCA could be demonstrated between patients with AIH and PBC as well as between patients with AIH and liver damage with uncertain causes(P<0.01).However,there was no significant difference between patients with PBC and liver damage with uncertain causes(P>0.05).The antigenic spectrums of ANCA in various groups of patients were as follows:In patients with AIH,the positive rates for lactoferrin,MPO,cathepsin G and BPI were 3%,11%,11% and 17% respectively.In patients with PBC,the positive rates for elastase,cathepsin G and BPI were 5%,5% and 16% respectively.In patients with liver damage of uncertain causes,the positive rate for BPI was 17%.It is apparent that in most patients with autoimmune disease,the presence of the atypical pANAC can be demonstrated in sera of these ptients with a positive detection rate of 28% to 70% and there is a concomitant presence of the characteristic autoantibodies.Thus,the detection of atypical pANAC might be helpful to the final diagnosis and classification of autoimmune liver diseases.