急进性肾小球肾炎英文课件.pptVIP

（3）EtiologyPathogenesisisunclear,environmentalfactorscausedtheexposureofGBMantigen?Beforeflu-likesymptoms：20%-60%FluvirusA2？Hydrocarbon---Inducedorexacerbated？汽油、柴油有机溶剂，油漆发胶杀虫剂强氧化剂如次氯酸装修(4)Predisposingfactorsmoke抗GBM抗体阳性者吸烟率稍高？发生肺出血机会大？造成肺泡内皮细胞通透性升高，抗原暴露？geneticsusceptibility孪生子发生率高，家族聚集现象HLA-DR2HLA-DRB1*1501HLA-DR15（5）PathogenesisAntiGBM-Abdirectparticipate抗体滴度与病情平行？动物模型以肾小球基底膜成分为抗原注射抗GBM抗体直接转移试验移植肾复发Alport综合症肾移植后TargetAntigenofantiGBM-Ab肾小球基底膜IV型胶原?3链非胶原区1（?3(IV)NC1）主要存在于GBM和TBM肺基底膜其它：眼、主动脉、脉络丛、耳蜗和神经肌肉接头等StructureofGBM(6)ClinicalmanifestationTwopeaks21-30yearsoldand51-70yearsold，genderdifferences？Onsetandmorehidden－UremiaattackorhemoptysisRaremulti-systemdamageMildhypertensionMoreperformancefortheRPGNpulmonaryhemorrhage72%-94%50%-80%beforenephritisAccompaniedbyDifficultybreathing：44%-72%Cough：18%-41%Smokemaybeinducelunghemorrhage;lunghemorrhageisusefulforearlydiagnose?Causingdeath!Youngman，antiGBM-Ab(+)，pulmonaryhemorrhageoccurredthedayaftersmokingYoungman，antiGBM-Ab(+)，Pulmonaryhemorrhageoccurredthedayaftersmoking，Suffocation，Autopsyshowed肺泡出血(Alveolarhemorrhage)，Inflammatorycellinfiltrationalveolarseptum,Hemosiderin-positivecells抗GBM抗体合并ANCA(IV型)发生率20%-35%临床表现可类似小血管炎ANCA阳性者应注意查抗GBM抗体治疗反应和预后取决于抗GBM抗体治疗前治疗后xx，F/21，Fever,jointpain,hemoptysisfor2months。cANCA(+)、antiGBM-Ab(+)肾功能正常的抗GBM抗体阳性患者!可达15%-36%表现为Goodpasture病肺出血轻重不等肾受累多表现为镜下血尿,肾活检为轻度系膜增生性肾小球肾炎抗GBM抗体多滴度低,阴转快.疗效肯定可能为早期表现?(7)LaboratoryexaminationHematuria,proteinuriaandNSisnotcommonGFRprogressivelydecreasedANCA(+)（1/4-1/3）Anemia78-100%30%ASO?,butnoevidenceofstreptococcalinfectionEarlyanti-GBMantibody(+)DetectofAntiGBM-Abdirectimmunofluorescence需要肾活检敏感性和特异性差不客观indirectimmunofluorescence新鲜冰冻肾组织敏感性和特异性差不客观ELISA-GBM可溶性抗原人或牛?(IV)NC1牛?3(IV)NC1Pathologicalexaminationlightmicroscope