抗线粒体抗体M2亚型阴性原发性胆汁性胆管炎的临床表现与生化指标分析

Clinical manifestations and biochemical indexes of patients with primary biliary cholangitis with negative anti-mitochondria antibody M2 subtype

目的:探讨抗线粒体抗体M2亚型(anti-mitochondria antibody M2 subtype,AMA-M2)阴性原发性胆汁性胆管炎(primary biliary cholangitis,PBC)患者的临床表现及生化指标特征。方法:收集2015年5月—2020年12月于南通大学附属南通第三医院住院的97例PBC患者的资料进行回顾性研究,以AMA-M2阴性者为观察组(27例),AMA-M2阳性者为对照组(70例),比较两组在一般情况及临床表现、实验室检测指标、肝脏循环免疫指标及病理学分期、熊去氧胆酸(ursodeoxycholic acid,UDCA)应答标准等方面上的差异。结果:两组患者在一般情况、临床表现、肝功能(谷丙转氨酶、谷草转氨酶、碱性磷酸酶、γ-谷氨酰转肽酶和总胆红素)、红细胞计数、血红蛋白、血小板、血小板分布宽度、总胆固醇、高密度脂蛋白和肝组织学表现上的差异均无统计学意义(均P>0.05)。AMA-M2阴性PBC患者血清免疫球蛋白M显著低于AMA-M2阳性患者,分别为2.36(0.49~8.38)g/L vs 2.94(0.90~6.67)g/L(P=0.043),而血清抗核抗体(antinuclear antibody,ANA)、抗gp210抗体阳性率高于AMA-M2阳性患者,分别为77.78%vs 40.00%和74.07%vs 51.43%(均P<0.05)。两组UDCA应答标准在巴黎Ⅰ、巴黎Ⅱ及鹿特丹标准上差异均无统计学意义(均P>0.05)。结论:AMA-M2阴性作为PBC的一个独特亚型,其一般情况、临床表现、肝功能指标、病理学表现、UDCA应答效果等方面与阳性者多无明显差异。临床诊断AMA-M2阴性PBC者,需通过联合免疫荧光法及免疫学检测以排除AMA-M2假阴性。抗gp210抗体、ANA、血清免疫球蛋白M值等可作为AMA-M2表达阴性患者诊断PBC的辅助指标。

Objective:To investigate the clinical manifestations and biochemical indexes of primary biliary cholangitis(PBC patients with anti-mitochondria antibody M2 subtype(AMA-M2)negative.Methods:The data of 97 PBC patients who were hospitalized in Nantong Third Hospital Affiliated to Nantong University from May 2015 to December 2020 were collected for retrospective study.27 patients with negative AMA-M2 expression were the observation group,and 70 patients with positive AMA-M2 expression were the control group.The differences of the two groups in general conditions and clinical manifestations,laboratory detection indexes,liver circulating immune indexes and pathological stages,ursodeoxycholic acid(UDCA)response criteria were compared.Results:There was no significant differences in general conditions,clinical manifestations,liver function(alanine aminotransferase,aspartate aminotransferase,alkaline phosphatase,γ-glutamyl transpeptidase and total bilirubin),red blood cell count,hemoglobin,platelets,platelet distribution width,total cholesterol,high-density lipoprotein and liver histological manifestations(all P>0.05).The serum immunoglobulin M in the AMA-M2 negative PBC patients was significantly lower than that in the AMA-M2 positive patients,2.36(0.49-8.38)g/L vs 2.94(0.90-6.67)g/L,P=0.043,while the positive rates of antinuclear antibody(ANA)and anti-nuclear pore membrane glycoprotein(gp210)antibody were higher than those of AMA-M2 positive patients,which were 77.78%vs 40.00%and 74.07%vs 51.43%(both P<0.05).There was no significant difference in the response effect of UDCA between the two groups on ParisⅠ,ParisⅡand Rotterdam standards(all P>0.05).Conclusion:AMA-M2 negative is a unique subtype of PBC,and its general conditions,clinical manifestations,liver function indicators,pathological manifestations,UDCA response effects and other aspects are not significantly different from those of positive ones.Clinical diagnosis of AMA-M2 negative PBC requires combined immunofluorescence and immunological testing to rule out AMA-M2 false negatives.Anti-gp210 antibody,ANA,serum immunoglobulin M value,etc.can be used as auxiliary indicators for the diagnosis of PBC in patients with negative AMA-M2 expression.