於强－血管炎病的诊断与治疗.ppt

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1、VasculitidesFudanuniversityZhongshanHospitalYuqiangThetermvasculitisreferstoaheterogeneousgroupofdisorderscharacterizedpathologicallybyevidenceofbloodvesselinflammationandclinicallybyadiversesetofsymptomsandsigns.Pathologythreetypes：1.necrotize2.granulomas3.onlyinflammatorycellul

2、arinfiltrationinthevesselwallCauseto1.vesselwallangusty，blunting、fibrosis，aneurysm2.tissueischemia，functionalimpaiementCLASSIFICATION1.histopathologic（1）necrotize（2）granulomas（3）leucocytoclasia（4）giantcell2.clinicalclassificinfectivityun-infectivity3.clinical+histopathologicprimari

3、lyskinsystemicsecondaryLieCLASSIFICATIONprimarilylarge-vesselTakayasuartertisgiantcellartertisMedium-vesselpolyarteritisnodosaChurg-StrusssyndromWegener’sgranulomatosissmall-vesselMicroscopicpolyangiitisSchonlein-HenochsyndromSecondaryinfectivitydrugconnectivetissuediseaseassociate

4、dwithtumerpathogenesis1.infectivity2.immunecomplex3.antineutrophilcytoplasmicantibodyANCA-PR3ANCA-MPO4.antibodyattackvesselAECAGBMantibody5.cellimmune6.understandclinicalmanifestationfeverdebllgeneralmalaise、anaemia、myosalgia、Prompt:feverrashjointpainmyosalgiaorganimpairedtheCha

5、pelHillConsensusConference1993giantcellartertis:Granulomatousarteritisoftheaortsandit’smajorbrancheswithapredilectionfortheextracranialbranchofthecarotidartery.Ofteninvolvedthetemporalartery.Usuallyoccuesinassociatedwithpolymyalgiarheumatica.Triad:1.templepain2.intermittencemandibular

6、movementdyskinesia3.acroiseageheadacheintermittencemandibularmovementdyskinesiatemporalarteritisscalppainbiopsy3/6TakayasuartertisGranulomatousinflammationoftheaortaanditsmajorbranches.Usuallyoccursinpatientsyoungerthen5o.Agelimpingimpulseattenuateddifferentialpressure>20mmHg

7、oisearteriography3/6polyarteritisnodosaNecrotizinginflammationofmedium-sizeorsmallarterieswithoutglomerulonephritisorvasculitisinarterioles,capilaries,orvenulesbodyweightdrawdown>4kglivedoreticularisorchialgiamyosalgiaNS.highBPCrBhepatitisarteriography3/10Microscopicp