狼疮性肾炎的病理-刘刚课件

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。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?OurworkRenalhistopathologicaldataof327patientswithrenalbiopsyprovenLNDiagnosedbetweenJanuary2000andJuly2008inPekingUniversityFirstHospital

Lupus.2009,18(12):1073-81.

IV-SIV-GPvalueNumberofpatients20152

Hemoglobin(mean±SD)(g/L)104.6±21.892.8±21.80.024Urineprotein(median;rangeMin-Max)(g/24hours)3.215,2-125.35,3-210.003Serumcreatinine(mean±SD)(mg/dL)1.0±0.272.07±2.050.021Creatinineclearancerate(mean±SD)(ml/min)81.29±19.7456.12±34.150.002Numberofanti-cardiolipinantibody(%)5/15(33.3)8/98(8.2)0.014C3(mean±SD)(g/L)0.46±0.190.38±0.160.045LabdataRenalOutcomeThefrequencyofserumANCAwassignificantlyhigherinIV-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.4/119KidneyInt.2009;76:307-317

KidneyInternationaladvanceonlinepublication,24February20105个单位协作313例，有2年以上随访资料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.TTP-HUSinLNTwelvepatientswithevidenceofTMAwereidentifiedin353patientswithLNSevenoutofthe12patientswerediagnosedasTTP-HUSLN+TTP-HUSLNPvalueNumberofpatients755Gender(male/female)1/610/451.0Age(mean±SD)(years)29.0±12.031.7±10.20.52Hemoglobin(mean±SD)(g/L)75.6±22.495.6±21.60.022NumberofLDHelevated(%)7(100)2(3.6)<0.001Numberofschistocytespresence(%)6(85.8)0(0)<0.001Numberofthrombocytopenia(%)7(100)8(14.5)<0.001Plateletcount(mean±SD)(x109/L)64.7±23.0161.9±72.20.001Acuterenalinjury(%)6(85.8)6(10.9)<0.001Serumcreatinine(mean±SD)(mg/dL)5.1±3.41.2±1.0<0.001ComparisonofclinicalandlaboratorydatabetweenpatientswithlupusnephritiswithandwithoutTTP-HUS

NephrolDialTransplant.2010,25:145TTP-HUSwasnotrareinpatientswithLNADAMTS-13autoantibodymightplayanimportantroleinthepathogenesisofTTP-HUSinLNTheirlong-termoutcomewaspoorercomparedwith“pure”LN

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(0,1)TMAA/A和/或TMAA/G(0,1)VAS(0,1)血管病变慢性病变评