抗肾小球基底膜抗体伴抗中性粒细胞胞浆.doc

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)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-neutrophil　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　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　C3　along　GBM.Tensive　therapy.Conclusion　Patients　with　coexisting　Anti-GBM　and　ANCA　tended　to　have　multi-system　involvement,similar　to　those　with　ANCA　positive　alone.Tgnosis　of　these　patients　was　poor,similar　to　patients　with　Anti-GBM　alone.　　【Key　words】　Crescentic　glomerul