狼疮性肾炎的病理-刘刚

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:MesangialproliferativeLGNIII:FocalLGN(involving50%ofglomeruli)A/CIV:DiffuseLGN(involving50%orglomeruli,IV-SandIV-G)A/CV: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-GStudiesParameterNajafiHillYokoyamaMittaletalHTNGSGSGSProteinuriaGSGSG≈SGSScrGSGSG≈SGSWireloopsGSGSGSSEdepositsGSGSIntInflamGSSGIntFibrosisG≈SGSSGAIGSGSGSSGCISGGSGSSGOutcomeSworseG≈S(ns)G≈S(ns)G≈S(ns)NajafietalKI2001,HilletalKI2005,YokohamaetalKI2004,MittaletalAJKD,2004OurworkRenalhistopathologicaldataof327patientswithrenalbiopsyprovenLNDiagnosedbetweenJanuary2000andJuly2008inPekingUniversityFirstHospitalLupus.2009,18(12):1073-81.IV-SIV-GPNumberofpatients20152Gender(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±5.310.962ClinicaldataIV-SIV-GPvalueNumberofpatients20152Hemoglobin(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±0.160.045LabdataIV-SIV-GPNumberofbiopsies20152Numberofglomeruli(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±0.340.001Interstitialinflammation(mean±SD)1.05±0.221.51±0.780.01CIscore(mean±SD)2.9±0.973.29±2.030.90PathologicaldataRenalOutcome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.3/119Althoughaggressiveimmunosuppressivetherapycouldachieveclinicalremission,theirlong-termrenaloutcomewaspoor.ESRD7/33vs.4/119KidneyInt.2009;76:307-317KidneyInternationaladvanceonlinepublication,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TwelvepatientswithevidenceofTMAwereidentifiedin353patientswithLNSevenoutofthe12patientswerediagnosedasTTP-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±1.00.001ComparisonofclinicalandlaboratorydatabetweenpatientswithlupusnephritiswithandwithoutTTP-HUSLN+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-HUSNephrolDialTransplant.2010,25:145TTP-HUSwasnotrareinpatientswithLNADAMTS-13autoantibodymightplayanimportantroleinthepathogenesisofTTP-HUSinLNTheirlong-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