醫(yī)學(xué)資料 腎病業(yè)務(wù)學(xué)習(xí)課件

Case24-year-old,maleMaincomplaintsRepeatedgrosshematuria

for4yrs

HistoryofPresentIllness:Theptsfoundhisurinearebrown,1dayafterupperrespirationinfection.thegrosshematuriacoulddisappearafterthetreatmentwithantibiotics.thesymptomrepeated1～2timesayear.Case

betweentheepisode,urineanalysis:persistentproteinuriaandhematuria(

Pro＋BLD3＋)Lab：IgG:576↓,IgA:142,IgE:<28.3C3:111,C4:27.8

BUN:13,Crea:0.6,albumin:3.9DPL:1911mg/day

renalbiopsy：IgAnephropathyWhatisIgAnephropathy?Whatisthereason

ofIgAN?WhyhegetsIgAN？HowtotreatIgAN?Whataboutit'sprognosis?IssueIgANephropathyDefinitionIgAmeansimmunoglobulinA,IgAnephropathy(IgAN)isakidneydisorderthatoccurswhenIgA—aproteinthathelpsthebodyfightinfections—settlesinthekidneys.IgAnephropathy

Firstreportedin1968，alsocalledBerger’sDiseasethecommonestprimaryGNvariable:

incidencearedifferentindifferentcountriesandraces

multiformityinclinicalmanifestation,andprognosisIgAnephropathyFinaldiagnosisisdependedon

ImmunofluorescencemicroscopyofrenalbiopsyGreatestfrequencyinCaucasiansandAsians.DiseaseprevalencemayinpartreflectregionalRenalbiopsypractices.Incidence

★SouthAmerica10%

★

Europe

20%

★

AsiaandPacific30-40%

★

China30%±m(xù)ale>femaleOccuratallagespredominate：olderchildrenandyoungadults,20+~30+y/o：↑thecommoncauseofCRFEtiologyMostcasesareidiopathicSLE,Sch?nlein-Henoch

SyndromLivercirrhosis.Glutenenteropathy（非熱帶性口炎性腹瀉）.HIVinfection.Wegener’sgranulomatosistreatedwithimmunosuppressiveagentsandURI(upperrespiratoryinfection).Familial.Dermatitisherpatiformis（皰疹性皮炎）

andseronegativearthritis（血清陰性關(guān)節(jié)炎）,

psoriasis

(牛皮癬).Gougerot-Houwesyndrome(干燥綜合征)PathogenesisUnknow:whatcausesIgAdepositstoforminthekidneys

.IgAnephropathymayruninfamiliesorberelatedtorespiratoryinfections.NoconsistenttriggerforthediseasehasbeenfoundAbnormalityofIgAregulationinresponsetoanenvironmentalantigen.PlasmaIgAconcentrationincreasedin50%ofcases.DirectcorrelationtocirculationIgAcomplexesanddiseaseseverity.IncreaseinIgA-specificBandTLymphocytesinresponsetoURI(upperrespiratoryinfection).Decreasedmucosalimmunity.IgAimmunecomplexactivatesalternatecomplementpathwaythuseliminationbymonocyte-macrophagesystemisslowandmorecomplexesareavailabletobedepositedontheglomerularmembrane.AlteredstructureofIgAcomplexesandelectrostaticchargealsoslowstheclearancemechanism.IgA-immunecomplexbindstofibronectinwhichhelpsinclearanceafterbindingwithuteroglobin.Abnormaluteroglobinorimpairedproductionpreventsclearance.PathologyLightmicroscopyfindingfocalordiffusemesangialproliferationandmatrixexpansion.Minorglomerularchang(20%),FSGS(28%),mesangialproliferativenephritis(50%),focalsegmentalglomerulonephritis,necrotizingglomerulonephritis,mesangiocapillary

glomerulonephritis,crescentic

glomerulonephritis,GlobulardepositsofIgA(alongwithC3andIgG)asdemonstratedbyImmunofluorescencemicroscopyinthemesangiumandglomerularcapillarywall.Electronmicroscopyshowingelectrondensedepositsofmesangium.Mesangialelectrondensedepositswith↑mesangialmatrix&cellularityinIgANClinicalPresentationClinicalfeature：1.predilectioninyoungmen：

predominate:13～15yr2.

manyptswithforerunnerinfectionbeforeepisodeorrelapse3.

hematuriaisverycommon一、hematuria：infectionortired40to50%presentwithrecurrentgrosshematuria.30to50%withmicroscopichematuriaandprotienuria.20to25%progresstogrosshematuria.二、proteinuriawithorwithoutmicroscopichematuria

thecommonestclinicalfeature(>50%)三、nephroticsyndrome

about3~4%，

areportinChina16.7％

四、Hypertension

AdultIgA>50%,children

5%。

about6yrsbeforerenalfailure。somepatientsmalignanthypertension五、Chronicrenalfailure10～20yrsafterdignosis，someptsatthefirstvisit。六、Acuterenalfailure（<10％）

1.ATN:RBCblockkidneytubules

2.Acutenephriticsyndrome，transientRF，pathologyissameasAGN3.Rapidlyprogressiveglomerulonephritis,

RPGN，progressiverenalfunctiondeteriorationFiveClinicalsyndromes1.Grosshematuria2.Asymptomaticmicroscopichematuriawith/withoutproteinuria----62%3.Acutenephritiswithhypertensionand/orrenalinsufficiency4.Nephroticsyndrome5.Amixednephritic-nephroticsyndromeLaboratoryexaminationUA:hematuria,proteinuriaserumIgA

↑(30～70%，China10～30％)

IgG，IgMarenormal，C3，CH50arenormalorslightly↑IgA-FN↑，IgA-IC↑someptswithHBsAgpositive

Diagnosisimmunohistochemicalfindingsof

mesangialdepositionofIgAlightmicroscopy:mesangialcell

proliferationisthecommonestfeatureDifferentialdiagnosis

1.post-streptococcalGN(AGN)latentperiodof10-21daysaffectchildrenprincipally,

hematuriaprincipally,hypertensiontransientrenalinsufficiencyCH50andC3lowbutnormalisesafter6-8weeks

2.chronicGN(non-IgA)

3.hereditaryGN

4.secondaryIgAN

Henoch-Sch?nleinpurpuraLupusnephritisHenoch-Sch?nleinpurpuraHenoch-Sch?nleinpurpura(HSP)HSP：clinicalsyndromeSame：histopathologicalalterationsDifference：HSPhassystemicsymptoms：purpuricrash,arthralgias,abdominalpain,acuteonset,self-limited.VariantsofthesamepathophysiologicprocessTreatmentGoal:preventprogressionofdiseaseandprotectrenalfunctionaccordingtotheclinicalfeatureandpathologictype

ACEInhibitorsCorticosteroidsImmunosuppressantsFish-oilsupplementTonsillectomy1、Hematuriaormildproteinuria

Forpatientswithnormalrenalfunction,

normotensionandonlyminorurinary

abnormalities,suchasisolatedmicroscopic

haematuria,and/ormildproteinuria,thegeneral

consensusisnottoofferspecifictreatmentbutto

keepsuchpatientsunderreview.

Upto23%ofpatientswillhaveaspontaneous

completeremission.TonsillectomyIgA：mucosadefensePopularinJapanandFrance.Indication：chronicinfections(dentalabscess,sinusitis)Inpediatric：tonsillectomy：↓grosshematuriaepisodes.NoevidenceforaffecttheprogressiontoCRIorESRD.Recommended：controversial2、massproteinuriaorNS3、Rapidlyprogressiveglomerulonephritis,RPGN

4、Chronicglomerulonephritistreattheinfectionantiplateletadhesiveness、anticoagulationeicosapentaenoicacid(一種魚油，3-ω脂肪酸)：postponeprogressofrenalfibrinolyticagent——urokinase，reduceth