狼疮性肾炎的病理刘刚.ppt

狼疮性肾炎的病理改变,北京大学第一医院肾内科刘刚,重点不在于诊断(但要注意合并其它病变)分型活动性和慢性化指标指导治疗和判断预后,肾脏病理,LN的病理分型,自1974年WHO首次公布LN的病理分型标准后，又分别在1982年、1995年及2003年进行了三次重大修订。,2003ISN/RPSConsensusConferenceontheClassificationofLupusNephritis(preliminary),I:Minimalmesangiallupusglomerulonephritis(LGN)II:MesangialproliferativeLGNIII:FocalLGN(involvingglomeruli,IV-SandIV-G)A/CV:MembranousLGN（可以与III或IV重叠）VI:AdvancedscleroticLGN(90%scleroticglomeruli),肾脏病理评分,活动指数慢性指数肾小球病变1.细胞增生1.肾小球硬化2.纤维素样坏死、核碎裂2.纤维性新月体3.细胞性新月体4.透明血栓，白金耳5.炎细胞浸润肾小管间质病变1.单核细胞浸润1.间质纤维化2.肾小管萎缩,Austinetal.1983,病理报告,狼疮性肾炎IV-G(A/C)V注明特殊病变新月体、纤维素样坏死等肾小管、肾间质、血管等,新分型带来的思考,ClassIV:IV-Svs.IV-G?,ComparisonofIV-SandIV-GStudies,ParameterNajafiHillYokoyamaMittaletalHTNGSGSGSProteinuriaGSGSGSGSScrGSGSGSGSWireloopsGSGSGSSEdepositsGSGSIntInflamGSSGIntFibrosisGSGSSGAIGSGSGSSGCISGGSGSSGOutcomeSworseGS(ns)GS(ns)GS(ns),NajafietalKI2001,HilletalKI2005,YokohamaetalKI2004,MittaletalAJKD,2004,Ourwork,Renalhistopathologicaldataof327patientswithrenalbiopsyprovenLNDiagnosedbetweenJanuary2000andJuly2008inPekingUniversityFirstHospital,Lupus.2009,18(12):1073-81.,Clinicaldata,Labdata,Pathologicaldata,RenalOutcome,ThefrequencyofserumANCAwassignificantlyhigherinIV-SgroupthanthatinIV-Ggroup(20%vs.4.6%,P=0.008)Thefrequenciesofanti-C1qIgG1andIgG3subclassweresignificantlyhigherinIV-GgroupthanthatinIV-Sgroup(P=0.006,P=0.011,respectively),CrGNwasnotrareinpatientswithLN（33/327）ANCAmightplayaroleincrescentformation.10/33vs.3/119Althoughaggressiveimmunosuppressivetherapycouldachieveclinicalremission,theirlong-termrenaloutcomewaspoor.ESRD7/33vs.4/119,KidneyInt.2009;76:307-317,KidneyInternationaladvanceonlinepublication,24February2010,5个单位协作313例，有2年以上随访资料,wefoundthatthe2003ISN/RPSclassificationsystemoflupusnephritis,basedonglomerularlesions,couldalsoreflectrelatedtubulointerstitiallesions.,141/313肾小球病变重、肾间质小管病变轻15/313肾小球病变轻、肾间质小管病变重,InmultivariateCoxhazardanalysisoftubulointerstitiallesions,indicesofinterstitialinfiltration,tubularatrophy,andinterstitialfibrosiswereconfirmedassignificantindependentriskfactorsforrenaloutcome.,小结,狼疮性肾炎病理分型尚需根据证据不断修订特殊病变可能具有独特的内在机制和临床特点肾间质小管病变对预后的影响更大,TTP-HUSinLN,TwelvepatientswithevidenceofTMAwereidentifiedin353patientswithLNSevenoutofthe12patientswerediagnosedasTTP-HUS,ComparisonofclinicalandlaboratorydatabetweenpatientswithlupusnephritiswithandwithoutTTP-HUS,ComparisonofrenalpathologicaldatabetweenpatientswithlupusnephritiswithandwithoutTTP-HUS,NephrolDialTransplant.2010,25:145,TTP-HUSwasnotrareinpatientswithLNADAMTS-13autoantibodymightplayanimportantroleinthepathogenesisofTTP-HUSinLNTheirlong-termoutcomewaspoorercomparedwith“pure”LN,正在进行的研究,研究对象：北京大学第一医院肾内科自2000年1月至2009年6月之间住院确诊的341例LN患者。入选标准：（1）依据1997年美国风湿病学会制定的SLE分类诊断标准，符合11项中4项或其以上者。（2）满足SLE的诊断标准，伴有持续的蛋白尿（0.5g/d或+）和/或管型尿，明确诊断为LN者。（3）在我院行肾活检并有完整的临床、病理及随访资料。（4）肾穿刺标本中肾小球数目10个，小动脉数目6个。ArthritisRheum.1997,病理评估：（1）病理分型及评分：按照2003年ISN/RPS制定的LN病理分型标准进行分型；按照NIH（NationalInstitutesofHealth）评分体系进行活动性和慢性化指标评分。KidneyInt.2004KidneyInt.1984,（2）肾血管病变评分LN血管病变类型VascularImmuneComplexDeposits(ICD)NoninflammatoryNecrotizingVasculopathy(NNV)ThromboticMicroangiopathy(TMA)TrueRenalVasculitis(TRV)Arteriosclerosis(AS)J.Am.Soc.Nephrol.1994,新AI和CI,血管病变活动性病变评分ICD(0,1)NNV(0