抗肾小球基底膜抗体伴抗中性粒细胞胞浆

抗肾小球基底膜抗体伴抗中性粒细胞胞浆

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时间:2018-05-25

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1、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)刘娜 赵明辉 章友康 王海燕  【摘要】 目的 了解抗肾小球基底膜抗体(Anti-GBM)伴抗中性粒细胞胞浆抗体(ANCA)阳性患者的临床病理特点。方法 对我科近4年来68例Anti-GBM和(或)ANCA阳性患者进行Anti-GBM及ANCA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-GBM阳性患者的24%,占全部ANCA阳性患者的7%。该4例患者Anti-GBM的百分结合率较单纯Anti-GBM阳性患者低。4例中3例髓过氧化物酶-ANCA(MPO-ANCA)

2、阳性,1例蛋白酶3-ANCA(PR3-ANCA)阳性,全身系统表现较多,与单纯性ANCA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IgG、C3呈细颗粒样分布于GBM。虽经积极治疗,多数患者预后较差,类似单纯Anti-GBM肾炎。结论 Anti-GBM伴ANCA阳性患者全身表现类似单纯ANCA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-GBM肾炎患者。  【关键词】 新月体肾炎 抗中性粒细胞胞浆抗体 抗肾小球基底膜抗体Anti-glomerular basement membrane antibodies coexisting with anti-n

3、eutrophil cytoplasmic autoantibodies.Report of 4 cases and review of literaturesLIU Na,ZHAO Minghui,ZHANG Youkang,et al.Institute of Nephrology,First Hospital of Beijing Medical University,Beijing 100034  【Abstract】 Objective To study patients with coexisting anti-glomerular basement membrane a

4、ntibodies(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.T

5、he 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 t

6、he 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 C3 along GBM.The prognosis of this type of patients was

7、 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 w

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