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目的 原发性胆汁性肝硬化 (PBC )的最重要特征为血清抗线粒体抗体 (AMA )阳性 ,但少数PBC病例AMA阴性。本文分析AMA阴性PBC患者的临床、生化和免疫学特性。方法 对 1991年 1月～ 2 0 0 2年 7月期间临床 /病理诊断PBC的 70例患者进行回顾性研究 ,分析比较AMA阴性和AMA阳性患者的一般资料、病程、临床表现、生物化学、免疫学及病理学等特征。结果 确诊PBC时 ,11例患者血清AMA/AMA M2 阴性 ,AMA阴性患者的血清免疫球蛋白M显著低于AMA阳性患者 ,分别为 (2 85 1± 14 18)mg/L和 (63 61± 492 8)mg/L ,P =0 .0 3 3 ,血清抗核抗体和 (或 )抗平滑肌抗体阳性率高于AMA阳性患者 (分别为 81.8%和 40 .7% ,P =0 .0 3 1) ,在年龄、性别比例、临床表现、肝功能试验、组织学表现和病理分期等各方面和AMA阳性患者无显著性差异。结论 AMA阴性PBC患者除血清免疫球蛋白M较低及抗核抗体和 (或 )抗平滑肌抗体阳性率较高外 ,其他临床特征和AMA阳性者相似
Purpose The most important characteristic of primary biliary cirrhosis (PBC) is serum anti-mitochondrial antibody (AMA) positive, but in a minority of PBC cases AMA negative. This article analyzes the clinical, biochemical and immunological characteristics of patients with AMA-negative PBC. Methods A retrospective study was conducted on 70 patients with clinically / pathologically diagnosed PBC between January 1991 and July 2002. The general data, course of disease, clinical manifestation, biochemistry and immunology of patients with AMA positive and AMA positive were analyzed. Learning and pathology and other characteristics. Results When PBC was diagnosed, serum AMA / AMA M2 was negative in 11 patients and serum immunoglobulin M in AMA negative patients was significantly lower than that in AMA positive patients (2 851 ± 14 18 mg / L and 63 61 ± 492, respectively) 8) mg / L, P = 0.303, the positive rate of serum antinuclear antibody and / or anti-smooth muscle antibody was higher than that of AMA positive patients (81.8% vs 40.7%, P = 0.031 ). There was no significant difference in age, sex ratio, clinical manifestations, liver function test, histological findings and pathological stage among patients with AMA positive. Conclusions AMA-negative PBC patients have similar clinical features and AMA positives except for lower serum immunoglobulin M and higher positive rate of antinuclear antibody and / or anti-smooth muscle antibody