伴免疫复合物沉积的ANCA相关性肺出血肾炎综合征
（附1例报道及相关文献复习）
肖慧捷杨霁云张焱刘景城
北京大学第一医院儿科100034
摘要目的:加强对抗中性粒细胞胞浆抗体ANCA（antineutrophilcytoplasmicantibody,ANCA）相关性小血管炎（ANCA－associatedsmallvasculitis,AASV）所致肺出血肾炎综合征的认识，探讨其发病机制、治疗和预后。方法:根据临床特征，血清ANCA抗体阳性、抗GBM抗体阴性、肾活检病理结果，除外了Goodpasture及其他系统性疾病。诊断为ANCA相关性小血管炎所致的肺出血肾炎综合征。并对相关文献进行复习。结果:本病例临床表现为贫血,肺出血、急性肾衰竭等多系统受累,肾穿刺活检可见广泛新月体形成并有毛细血管袢纤维素样坏死,血清学检查ANCA阳性,并除外了GBM抗体相关的肺出血肾炎综合征（Goodpasture）及系统性红斑狼疮等其它系统性疾病。但荧光检查却有免疫复合物在GBM、系膜区颗粒及短线状沉积（免疫球蛋白≥＋＋）。与经典的AASV所致肺出血肾炎综合征的免疫荧光呈阴性或弱阳性不同。结论:AASV所致的肺出血肾炎综合征确诊主要依靠ANCA血清学检测和经皮肾穿刺活检病理,并除外其他相关的疾病。但经典的AASV所致的肺出血肾炎综合征免疫荧光呈阴性或弱阳性,本例为国内首例儿童有免疫沉积型的以肺出血肾炎综合征为临床表现的AASV的报道。治疗主要为肾上腺皮质激素和免疫抑制剂。ANCA试验及肾活检有利于早期诊断。早期积级治疗本病,可有效改善预后。AASV中免疫球蛋白在肾小球阳性沉积的意义仍有待于进一步积累资料。
关键词 抗中性粒细胞胞浆抗体ANCA;ANCA相关性小血管炎;小儿
ImmunecomplexdepositsinANCA-associatedpulmonary-renalsyndrome:acasereportandliteraturereview
XIAOHuijie,YANGJiyun,ZHANGYan,LIUJingcheng,
DepartmentofPediatric,PekingUniversityFirstHospital,Beijing,100034
Toimprovetheknowledgeofantineutrophilcytoplasmicantibody（ANCA）associatedwithsmallvasculitis（AASV）ofpulmonary-renalsyndrome,withimmunecomplexdepositionwithinglomerulionimmunofluorescence,analysetheclinicalandpathologicalfeaturesofANCAassociatedAASVinchildren.
METHODAccordingtotheclinicalfeatures,positiveANCAserologytests,negativeGBMantibody,renalbiopsyfindings,excludedtheGoodpasture'sSyndrome,andotherunderlyingsystemicdisease，thediagnosiswasmade.Relateddatawerereviewed.comparewiththatofclassical“pauci-immune”pulmonary-renalsyndrome.
RESULTThe11-year-oldgirlpresentedwithanemia,diffusepulmonaryhemorrhageinconjunctionwithrapidlyprogressiveglomerulonephritis,etc.Therenalbiopsydisplaysfocalsegmentalfibroidnecrosisoftheglomerularcapillarytuftsanddiffusecrescentsformation,withimmunecomplexdeposits.
CONCLUSIONWereportacaseofANCA-associatedsystemicvasculitiswithimmunecomplexdeposition.AASVisasystemicdisease,necrotizingandcrescenticglomerulonephritisrelatedtoANCAassociatedpulmonary-renalsyndromeistypicallyreferredtoas“pauci-immune”.Pulmonary-renalsyndromecanoriginatefromvarioussystemicautoimmunediseases.ANCA-associatedvasculitidesaccountforapproximately60%,Goodpasture'sSyndromeforapproximately20%ofthecases.Renalbiopsydisplaysextracapillaryproliferatingglomerulonephritis
inwhichtheclinicalandpathologicalfeatureswereextracapillaryproliferatingglomerulonephritis,focalsegmentalfibroidnecrosisoftheglomerularcapillarytuftsanddiffusecrescentsformation,Reportsof
Intenseimmunosuppressionwithcyclophosphamideandglucocorticoids,.EarlydiagnosisandappropriateintensivetherapyarecriticalforpatientswithidiopathicRPGN.Thedegreeofrenalfunctionandthepercentofcrescentsonrenalbiopsyarebetterpredictorsofoutcome.PediatricpatientswithAASVwerenotrareinChina.TheclinicalandpathologicalfeaturesofpatientswithAASVinchildhoodweresimilartoadultpatients.
Keywords:antineutrophilcytoplasmicantibody（ANCA）;
ANCAassociatedsystemicvasculitis（AASV）;child,
mainlydependontheserumofANCAtolearntheexaminationand
