狼瘡性腎炎的病理改變

北京大學(xué)第一醫(yī)院腎內(nèi)科劉剛精選課件

重點不在于診斷(但要注意合并其它病變)分型活動性和慢性化指標指導(dǎo)治療和判斷預(yù)后

腎臟病理精選課件LN的病理分型自1974年WHO首次公布LN的病理分型標準后，又分別在1982年、1995年及2003年進行了三次重大修訂。精選課件2003ISN/RPSConsensusConferenceontheClassificationofLupusNephritis(preliminary)I:Minimalmesangiallupusglomerulonephritis(LGN)II:MesangialproliferativeLGNIII:FocalLGN(involving<50%ofglomeruli)A/CIV:DiffuseLGN(involving50%or>glomeruli,IV-SandIV-G)A/CV:MembranousLGN（可以與III或IV重疊）VI:AdvancedscleroticLGN(>90%scleroticglomeruli)精選課件腎臟病理評分

活動指數(shù)慢性指數(shù)腎小球病變

1.細胞增生 1.腎小球硬化

2.纖維素樣壞死、核碎裂 2.纖維性新月體

3.細胞性新月體

4.透明血栓，白金耳

5.炎細胞浸潤腎小管間質(zhì)病變

1.單核細胞浸潤 1.間質(zhì)纖維化

2.腎小管萎縮

Austinetal.1983精選課件病理報告狼瘡性腎炎IV-G(A/C)＋V注明特殊病變新月體、纖維素樣壞死等腎小管、腎間質(zhì)、血管等精選課件新分型帶來的思考精選課件ClassIV:IV-Svs.IV-G?精選課件ComparisonofIV-SandIV-GStudiesParameter Najafi Hill Yokoyama MittaletalHTN G>S G>S G>SProteinuria G>S G>S G≈S G>SScr G>S G>S G≈S G>SWireloops G>S G>S G>SSEdeposits G>S G>SIntInflam G>S S>GIntFibrosis G≈S G>S S>GAI G>S G>S G>S S>GCI S>G G>S G>S S>GOutcomeSworse G≈S(ns)G≈S(ns) G≈S(ns) NajafietalKI2001,HilletalKI2005,YokohamaetalKI2004,MittaletalAJKD,2004精選課件OurworkRenalhistopathologicaldataof327patientswithrenalbiopsyprovenLNDiagnosedbetweenJanuary2000andJuly2008inPekingUniversityFirstHospital

Lupus.2009,18(12):1073-81.精選課件

IV-SIV-GP

Numberofpatients20152Gender(male/female)1/1926/1260.207Age(mean±SD)(years)34.1±10.931.3±10.60.277Numberoforalulcer(%)11(55)40(26.3)0.017Numberofanemia(%)12(60)135(88.8)0.003Numberofhematuria(%)15(75)144(94.7)0.009Numberofnephroticsyndrome(%)8(40)109(71.1)0.009Acutekidneyinjury(%)1(5)57(37.5)0.002SLEDAI(mean±SD)19.4±5.4719.46±5.310.962Clinicaldata精選課件

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±0.160.045Labdata精選課件

IV-SIV-GPNumberofbiopsies20152

Numberofglomeruli(mean±SD)32.3±14.328.2±11.50.148%ofcaseswithfibrinoidnecrosis25(5/20)4.6(7/152)0.006AIscore(mean±SD)8.75±2.7310.93±3.360.006Endocapillaryhypercellualrity(mean±SD)2.35±0.592.92±0.34<0.001Interstitialinflammation(mean±SD)1.05±0.221.51±0.780.01CIscore(mean±SD)2.9±0.973.29±2.030.90Pathologicaldata精選課件RenalOutcome精選課件ThefrequencyofserumANCAwassignificantlyhigherinIV-SgroupthanthatinIV-Ggroup(20%vs.4.6%,P=0.008)Thefrequenciesofanti-C1qIgG1andIgG3subclassweresignificantlyhigherinIV-GgroupthanthatinIV-Sgroup(P=0.006,P=0.011,respectively)

精選課件CrGNwasnotrareinpatientswithLN（33/327）ANCAmightplayaroleincrescentformation.10/33vs.3/119Althoughaggressiveimmunosuppressivetherapycouldachieveclinicalremission,theirlong-termrenaloutcomewaspoor.ESRD7/33vs.4/119KidneyInt.2009;76:307-317

精選課件KidneyInternationaladvanceonlinepublication,24February2010精選課件5個單位協(xié)作313例，有2年以上隨訪資料精選課件wefoundthatthe2003ISN/RPSclassificationsystemoflupusnephritis,basedonglomerularlesions,couldalsoreflectrelatedtubulointerstitiallesions.精選課件141/313腎小球病變重、腎間質(zhì)小管病變輕15/313腎小球病變輕、腎間質(zhì)小管病變重精選課件InmultivariateCoxhazardanalysisoftubulointerstitiallesions,indicesofinterstitialinfiltration,tubularatrophy,andinterstitialfibrosiswereconfirmedassignificantindependentriskfactorsforrenaloutcome.精選課件小結(jié)狼瘡性腎炎病理分型尚需根據(jù)證據(jù)不斷修訂特殊病變可能具有獨特的內(nèi)在機制和臨床特點腎間質(zhì)小管病變對預(yù)后的影響更大精選課件TTP-HUSinLNTwelvepatientswithevidenceofTMAwereidentifiedin353patientswithLNSevenoutofthe12patientswerediagnosedasTTP-HUS精選課件LN+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

精選課件LN+TTPLNPvalueNumberofbiopsies755IV-G(A)/IV-G(A/C)2/521/341.0Activityindex(AI)score11.7±3.110.9±2.80.494Endocapillaryhypercellualrity3±03.0±0.130.724Cellularcrescents3.14±2.262.11±1.700.149Karyorrhexis/fibrinoidnecrosis1.29±0.951.36±0.930.836Subendothelialhyalinedeposits1.0±0.581.8±1.150.075Interstitialinflammation2.14±0.691.4±0.830.027Leukocyteinfiltration1.14±1.071.29±0.830.669Chronicityindex(CI)score4.86±2.542.71±1.760.005Glomerularsclerosis1.0±1.00.32±0.580.01Fibrouscrescents0.14±0.380.13±0.390.92Tubularatrophy1.86±0.901.18±0.640.015Interstitialfibrosis1.88±0.901.07±0.690.008ComparisonofrenalpathologicaldatabetweenpatientswithlupusnephritiswithandwithoutTTP-HUS

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

精選課件正在進行的研究研究對象：北京大學(xué)第一醫(yī)院腎內(nèi)科自2000年1月至2009年6月之間住院確診的341例LN患者。入選標準：

（1）依據(jù)1997年美國風濕病學(xué)會制定的SLE分類診斷標準，符合11項中4項或其以上者。

（2）滿足SLE的診斷標準，伴有持續(xù)的蛋白尿（>0.5g/d或>+++）和/或管型尿，明確診斷為LN者。

（3）在我院行腎活檢并有完整的臨床、病理及隨訪資料。

（4）腎穿刺標本中腎小球數(shù)目≥10個，小動脈數(shù)目≥6個。

ArthritisRheum.1997精選課件病理評估：

（1）病理分型及評分：按照2003年ISN/RPS制定的LN病理分型標準進行分型；按照NIH（NationalInstitutesofHealth）評分體系進行活動性和慢性化指標評分。

KidneyInt.2004KidneyInt.1984精