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1、关于大动脉炎(LVV)科内文献汇报:孙闻嘉2015.11我的问题1.基本概念:LVV定义和分型?2.LVV的评估?3.LVV的治疗?1.基本概念:LVV定义和分型?2.LVV的评估?3.LVV的治疗?TAKvs.GCATAKGCAage<50yrs>50yrsraceAsianCaucasiangeneHLA-B*52.HLA-B/MICA,HLA-DQB1/HLA-DRB1,FCGR2A/FCGR3AHLA-DRB1*04typeTimetodiagnosisdelayedOutcomemeasuresBVASan
2、dVasculitisDamageIndex(VDI)???CRP,ESR,andcharacteristicsymptomsTAKtypetype1:branchesoftheaorticarch;type2a,ascendingaorta,aorticarchanditsbranches;type2b,ascendingaorta,aorticarchanditsbranchesandthoracicdescendingaorta;type3,descendingthoracicaorta,abdominalao
3、rtaand/orrenalarteries;type4,abdominalaortaand/orrenalarteries;type5,combinedfeaturesoftypes2band4.inaddition,involvementofthecoronaryandpulmonaryarteriesshouldbeindicated.分型的意义在于???图TAK分型的意义?1.基本概念:LVV定义和分型?2.LVV的评估?3.LVV的治疗?(1)目前用于临床研究的评估手段(2)临床上怎么评估?(3)影像学评估
4、临床研究的评估手段(1)血管炎活动度评估:1994theBVASv3/WG,2001DiseaseExtentIndex(DEI),血管炎损害评估:1997VDI2004combineddamageassessment(CDA)临床研究的评估手段(2)生活质量评估(QOL):SF-36EuroQolfive-dimensionsquestionnaire(EQ-5D)nocorrelationsbetweenSF-36scoresandangiographictype,diseaseduration,ESRorCRP
5、levelsintheTAKpatients.AnegativecorrelationwasobservedbetweentheVDIandSF-36scores临床研究的评估手段(3)TAK-specificdiseaseactivitymeasures1994NIH2004DEI-Takayasu(DEI.Tak)2006IndianTakayasuClinicalActivityScore(ITAS2010)TAK-specificdamagemeasures临床上怎么评估?imageFDG-PETMRIUSC
6、T1.分析包括21项研究,413例LVV,127例GCA,197例TAK2.Control:299名患者,226例肿瘤,31例其他(感染18,RA6,AAV5),44例不详3.156/416:接受治疗4.PositicePET:usingauthors’criteriaPET用于GCA诊断:敏感性0.89,特异性0.98,且研究一致性好PET用于TAK活动性评估:敏感性0.86,特异性0.73,且研究一致性差PET用于TAK活动性评估:均用NIHscale评估活动度。敏感性0.860.84,特异性0.730.84.P
7、ET用于TAK活动性评估与其他指标的一致性:2.Significantvascularuptake(moderateandhigh)inNomarkersofactivityvs.3/3markersofactivity:8/12vs.14/16(67%vs.88%)P值VisualgradingSUVmaxratioclinicalactivity0.010.01CRP0.040.0006ESR0.040.02imageFDG-PETMRA:forstructureandwalledemaActive/inacti
8、ve:sen100%,spe89%;PPV92%,NPV100%.(???)MRArevealwalledema:active94%vs.inactive56%,且与ESR及CRP无关sustainedvesselwalloedemareflectsbothactivityanddamage,althoughvesselwalloedemaoc