AMA/AMA-M2阴性原发性胆汁性肝硬化患者的临床及病理特征  

Clinical and pathological features of primary biliary cirrhosis in anti-mitochondria antibody/anti-mitochondrial antibody M2 subtype negative patients

作　　者：刘卫英[1] 肖琳[1] 银皓[1] 詹欣宇[1] 张泽高[1] 鲁晓擘[1] 张跃新[1] 

机构地区：[1]新疆医科大学第一附属医院感染科,新疆维吾尔自治区乌鲁木齐市830054

出　　处：《世界华人消化杂志》2013年第34期3799-3805,共7页World Chinese Journal of Digestology

摘　　要：目的:通过探讨抗线粒体抗体(anti-mitochondrial antibody,AMA)/AMA-M2阴性原发性胆汁性肝硬化(primary biliary cirrhosis,PBC)患者的临床表现及肝脏组织学病理特征,提高对PBC的认识和诊断水平.方法:回顾性分析101例PBC患者的临床表现、生物化学、免疫学及病理学资料.比较50例AMA/AMA-M2阴性和51例AMA/AMA-M2阳性PBC患者的临床和病理学特征.结果:101例PBC患者中,男女比例为1.1∶9,平均年龄为48.52岁±8.99岁,临床表现为乏力(30.7%)、黄疸(29.7%)、皮肤瘙痒(19.8%)、口干(12.9%)、眼干(7.9%)、肝区疼痛(7.9%)、脂黄瘤(6.9%)、纳差(5.9%)、腹胀(1.98%)等.AMA/AMA-M2阴性PBC和AMA/AMA-M2阳性PBC患者的临床表现、血常规[白细胞(white blood cells,WBC)、血红蛋白(hemoglobin,Hb)、红细胞(red blood cells,RBC)、血小板(platelet,PLT)]、生化指标[门冬氨酸氨基转氨酶(aspartate aminotransaminase,AST)、丙氨酸氨基转氨酶(alanine aminotransferase,ALT)、AST/ALT、碱性磷酸酶(alkaline phosphatase,ALP)、-谷氨酰胺肽酶(glutamine peptide enzyme,GGT)、总胆红素(total bilirubin,TBIL)、总蛋白(total protein,TP)、白蛋白(albumin,ALB)、甘油三酯(triglycerides,TG)、总胆固醇(total cholesterol,TC)]、凝血指标[凝血酶原活动度(prothrombin activityprothrombin time activity,PTA)、国际标准化比值(international normalized ratio,INR)]、C3、C4、影像学和肝脏组织学表现上差异均无统计学意义.AMA/AMA-M2阳性PBC组与AMA/AMA-M2阴性PBC组比较,-球蛋白、IgG、IgM差异具有统计学意义(F=0.019,Z值分别为0.028,0.012,均P<0.05).结论:AMA/AMA-M2阴性的PBC患者临床表现缺乏特异性,AMA/AMA-M2阳性PBC患者与AMA/AMA-M2阴性PBC患者免疫状态可能存在差异.肝脏组织病理学检查仍然是诊断AMA/AMA-M2阴性PBC的金标准.AIM： To improve the level of awareness and diagnosis of primary biliary cirrhosis （PBC） by exploring the clinical and pathological features of PBC in patients who are negative for anti-mi- tochondria antibody （AMA）/anti-mitochondrial antibody M2 subtype （AMA-M2）. METHODS： A retrospective study was carried out in 101 patients with PBC. Clinical manifesta- tions, biochemical, immunological and histo- pathological data were analyzed. The clinicaland pathological features were compared be- tween 50 AMA/AMA-M2 negative pati.ents and 51 AMA/AMA-M2 positive patients. RESULTS： The ratio of male to female in the 101 patients with PBC was 1.1：9, and the mean age at diagnosis was 48.52 ±8.99 years. Common manifestations were fatigue （30.7%）, jaundice （29.7%）, pruritus （19.8%）, dry mouth （12.9%）, dry eye （7.9%）, hepatalgia （7.9%）, lipomyoma （6.9%）, anorexia （5.9%）, and abdominal distension （1.98%）. There were no significant differences in manifestation between the AMA/AMA-M2 negative group and AMA/AMA-M2 positive group. There were also no significant differences in routine blood indices [white blood cells （WBC）, hemoglobin （Hb）, red blood cells （RBC）, and platelet （PLT）], biochemical indices [aspartate aminotransferase （AST）, alanine aminotransferase （ALT）, AST/ALT, alkaline phosphatase （ALP）, gamma glutamine transpeptidase （GGT）, total bilirubin （TBIL）, total protein （TP）, albumin （ALB）, triglycerides （TG）, and total cholesterol （TC）], coagulation function [prothrombin time activity （PTA）, international normalized ratio （INR）], C3, C4, and histopathology between the two groups. γ-globulin, IgG and IgM in the AMA/AMA-M2 positive group were significantly higher than those in the AMA/AMA-M2 negative group （F = 0.019, Z = 0.028, 0.012, P 〈 0.05）. CONCLUSION： AMA/AMA-M2 negative PBC has no specific clinical manifestations, There may be difference in immune state between the AMA/ AMA-M2 positive group and

关 键 词：肝硬化 胆汁性 病理学 临床特点 抗线粒体抗体 抗线粒体抗体-M2 

分 类 号：R575.2[医药卫生—消化系统]