肾内学习一快速进展性igan

IgAnephropathyexistsinthreeformsi)lanthanic(covert)—onlybyarenalbiopsyinanotherwisehealthyperson(suchasa"zero“timebiopsyofadonorkidney);ii)clinicallysilent—onlyfoundwhenaroutineurinalysisisperformedinanasymptomaticindividual(suchasapre-employmentorpre-insurancephysicalexaminationrevealingmicroscopichematuriaand/ororlow-gradeproteinuria)andarenalbiopsyisperformedtoconfirmadiagnosis;iii)clinicallyovert—diagnosedwhenarenalbiopsyperformedinapatientwithgross(visible)hematuriaand/orproteinuria,withorwithouthypertension.OxfordClassificationofIgAN:

没有包括新月体型TheOxfordclassificationofIgAnephropathy(IgAN)includes：mesangialhypercellularity(M),endocapillaryhypercellularity(E),segmentalglomerulosclerosis(S),andtubularatrophy/interstitialfibrosis(T)extracapillaryproliferation(Ex)wasnotaddressed.肾活检时urinaryprotein0.5g/dandeGFR30ml/minper1.73m2被除外Kidneysurvivalcurveinpatientswithandwithoutextracapillaryproliferationinthepatientswhodidnotmeetinclusion

criteriaoftheOxfordclassificationClinJAmSocNephrol6:2806–2813,December,2011WithadditionofEx,notSbutExwassignificantforESRF.Haas分型系统IgAN-MHTwasnotassociatedwiththebackgroundglomerularpathologicalphenotypesofIgAN45patientswithIgAN-MHT,7(15.56%),5(11.11%),13(28.89%),9(20%)and11(24.44%)patientsweregradedasHassI,II,III,IVandVseverityofnon-ischaemicsclerosis,crescentsandmesangialproliferationweresignificantlydifferentbetweenpatientswithdifferentgradesBloodpressure,SCrandproteinuriaatpresentationwerecomparableNephrolDialTransplant(2008)23:3921–3927RenalandpatientsurvivalinmalignanthypertensionsecondarytoIgAnephropathy(IgANMHT)patientsandprimarymalignanthypertension(PMHT)patientsKidneyBloodPressRes2005;28:251–258IgA肾病合并TMAIgA肾病常常合并肾内小动脉或者细小动脉病变，表现为管壁增厚和玻璃样变。合并TMA的IgA肾病71%存在难以控制的高血压，其中26%为恶性高血压。肾脏组织中，硬化的肾小球与明显的肾小管间质病变。Thromboticmicroangiopathy(TMA)Aheterogeneousdisordercharacterizedbyplateletthrombiinarteriolesand.RenalhistopathologiclesionsinTMAtendtotakeoneoftwobroadformswithconsiderableoverlap:(1)predominantarteriolar,andlesserarterial,involvement,withthrombiandfibrinoidnecrosis,particularlyinTTP,malignanthypertension(MHT),andscleroderma;(2)glomerularinvolvement,withcapillarythrombi,capillaryloopswithdoublecontoursduetomesangialinterposition,andvariablemesangiolysis,thelattermostfrequentlyseenintheHUS.HistologicFindingsinPatientswith

IgAN-AssociatedTMAAcuteTMAinartery.FreshTMAinsegmentofinterlobularartery(arrow,upperright).Adjacentsectionshowsmarkedintimalfibroplasiasandmildmedialhypertrophy.Patientwithmalignanthypertension.OrganizingTMAinafferentarteriole.Residualredstainingfibrinoidmaterial,withportionoffoamcellvisible.Internalelasticaismaximallystretched.Glomerulusandparenchymalargelyintact.Patientormotensive,withoutantihypertensiveagents.JAmSocNephrol23,2012.doi:10.1681/ASN.2010111130Significanceofdifferencesinsurvivalbetween

groupsJAmSocNephrol23,2012.doi:10.1681/ASN.2010111130markersofpooreslongevityofmacroscopichematuriaage>50yearsdecreasedbaselineeGFRabsenceofpreviousepisodesofmacrohematuriaseveretubularnecrosisClinJAmSocNephrol2007;2:51–7.Mainpathohisto