抗肾小球基底膜抗体伴抗中性粒细胞胞浆抗体阳性肾炎(四例报告及文献复习  

Anti glomerular basement membrane antibodies coexisting with anti neutrophil cytoplasmic autoantibodies.Report of 4 cases and review of literatures

作　　者：刘娜[1] 赵明辉[1] 章友康[1] 王海燕[1] 

机构地区：[1]北京医科大学第一医院肾内科肾脏病研究所,100034

出　　处：《中华肾脏病杂志》1999年第4期252-254,共3页Chinese Journal of Nephrology

摘　　要：目的　了解抗肾小球基底膜抗体（ Ａｎｔｉ Ｇ Ｂ Ｍ） 伴抗中性粒细胞胞浆抗体（ Ａ Ｎ Ｃ Ａ） 阳性患者的临床病理特点。方法　对我科近４ 年来６８ 例 Ａｎｔｉ Ｇ Ｂ Ｍ 和（ 或） Ａ Ｎ Ｃ Ａ 阳性患者进行 Ａｎｔｉ Ｇ Ｂ Ｍ 及 Ａ Ｎ Ｃ Ａ 检测，对其中４ 例两者均阳性患者进行临床病理分析。结果　６８ 例患者中４ 例两者均阳性，占全部 Ａｎｔｉ Ｇ Ｂ Ｍ 阳性患者的２４ ％ ，占全部 Ａ Ｎ Ｃ Ａ 阳性患者的７ ％ 。该４ 例患者 Ａｎｔｉ Ｇ Ｂ Ｍ 的百分结合率较单纯 Ａｎｔｉ Ｇ Ｂ Ｍ 阳性患者低。４ 例中３ 例髓过氧化物酶 Ａ Ｎ Ｃ Ａ（ Ｍ Ｐ Ｏ Ａ Ｎ Ｃ Ａ） 阳性，１ 例蛋白酶３ Ａ Ｎ Ｃ Ａ（ Ｐ Ｒ３ Ａ Ｎ Ｃ Ａ） 阳性，全身系统表现较多，与单纯性 Ａ Ｎ Ｃ Ａ 阳性患者相似。所检病例肾脏病理多为新月体肾炎，免疫荧光检查多为 Ｉｇ Ｇ、 Ｃ３ 呈细颗粒样分布于 Ｇ Ｂ Ｍ。虽经积极治疗，多数患者预后较差，类似单纯 Ａｎｔｉ Ｇ Ｂ Ｍ 肾炎。结论　 Ａｎｔｉ Ｇ Ｂ Ｍ 伴 Ａ Ｎ Ｃ Ａ 阳性患者全身表现类似单纯 Ａ Ｎ Ｃ Ａ 相关小血管炎患者，但治疗效果及预后相对较差又类似于 Ａｎｔｉ Ｇ Ｂ ＭObjective To study patients with coexisting anti glomerular basement membrane antibodies(Anti GBM)and anti neutrophil cytoplasmic autoantibodies(ANCA),and to analyze their clinical and pathological features.Methods 68 sera from 68 patients with Anti GBM and/or ANCA(MPO or PR3 ANCA)related diseases were collected and were further tested for Anti GBM and ANCA respectively.The clinical and pathological features of patients with coexisting Anti GBM and ANCA were analyzed.Results Of the 17 sera with Anti GBM,55 sera wtih ANCA,4 sera had both Anti GBM and ANCA (3 MPO ANCA,1 PR3 ANCA),which represented approximately 24% of the Anti GBM positive population and 7% of the ANCA positive patients.Of the 4 patients with both antibodies,they tended to have multi system involvement.Renal pathology showed crescentic glomerulonephritis,direct immunofluorescence demonstrated fine granular localization of IgG and C 3 along GBM.The prognosis of this type of patients was poor despite intensive therapy.Conclusion Patients with coexisting Anti GBM and ANCA tended to have multi system involvement,similar to those with ANCA positive alone.The prognosis of these patients was poor,similar to patients with Anti GBM alone.

关 键 词：肾炎 Anti-GBM ANCA 病例报告 新月体肾炎 

分 类 号：R692.39[医药卫生—泌尿科学]