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1、抗腎小球基底膜抗體伴抗中性粒細胞胞漿抗體陽性腎炎(四例報告及文獻復習)劉娜 趙明輝 章友康 王海燕 【摘要】 目的 了解抗腎小球基底膜抗體(Anti-GBM)伴抗中性粒細胞胞漿抗體(ANCA)陽性患者的臨床病理特點。方法 對我科近4年來68例Anti-GBM和(或)ANCA陽性患者進行Anti-GBM及ANCA檢測，對其中4例兩者均陽性患者進行臨床病理分析。結(jié)果 68例患者中4例兩者均陽性，占全部Anti-GBM陽性患者的24%，占全部ANCA陽性患者的7%。該4例患者Anti-GBM的百分結(jié)合率較單純Anti-GBM陽性患者低。4例中3例髓過氧化物酶-ANCA(MPO-ANCA)
2、陽性，1例蛋白酶3-ANCA(PR3-ANCA)陽性，全身系統(tǒng)表現(xiàn)較多，與單純性ANCA陽性患者相似。所檢病例腎臟病理多為新月體腎炎，免疫熒光檢查多為IgG、C3呈細顆粒樣分布于GBM。雖經(jīng)積極治療，多數(shù)患者預后較差，類似單純Anti-GBM腎炎。結(jié)論 Anti-GBM伴ANCA陽性患者全身表現(xiàn)類似單純ANCA相關小血管炎患者，但治療效果及預后相對較差又類似于Anti-GBM腎炎患者。 【關鍵詞】 新月體腎炎 抗中性粒細胞胞漿抗體 抗腎小球基底膜抗體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
