抗线粒体抗体阴性原发性胆汁性肝硬化的临床特征

Clinical evaluation of serum anti-mitochondrial antibody-negative primary biliary cirrhosis

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 。

Objective Primary biliary cirrhosis (PBC) is characterized by frequent presence of anti mitochondrial antibody (AMA). To evaluate whether or not the presence of AMA alters the clinical, serological and histological features of the disease. Methods Clinical data of 70 patients clinically and/or histologically diagnosed with PBC were reviewed. AMA negative and AMA positive patients were compared in terms of clinical, biochemical, immunological and histological features. Results At presentation, 11 patients were serum AMA/AMA M 2 negative. At the initial visit, AMA negative and AMA positive patients were similar in terms of age, sex, clinical manifestations, liver biochemistries and histological findings. Mean serum immunoglobulin M (lgM) level was significantly lower in AMA negative PBC patients when compared with AMA positive PBC patients (2 851±1 418mg/L vs. 6 361±4 928mg/L, P =0.033). Serum anti nuclear antibodies (ANA) and/or anti smooth muscle antibodies (ASMA) were more frequently positive in the AMA negative PBC patients than in the AMA positive patients (81.8% vs. 40.7%, P =0.031).Conclusions AMA negative PBC patients are characterized by relatively lower serum lgM levels and a higher prevalence of serum ANA/ASMA and are not associated with substantial differences in the clinical, biochemical and histological spectrum of the disease.