大动脉炎并不比小血管炎简单课件.pptx

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？TAK vs. GCATAKGCAage 50yrs 50yrsraceAsianCaucasiangene HLA-B*52 . HLA-B/MICA, HLA-DQB1/HLA-DRB1, FCGR2A/FCGR3AHLA-DRB1*04typeTime to diagnosis delayedOutcome measures BVASand Vasculitis Damage Index (VDI) ??? CRP, ESR, and characteristicsymptomsTAK type分型的意义在于？？？ 图 type 1: branches of the aortic arch; type 2a, ascending aorta,aortic arch and its branches; type 2b, ascending aorta, aortic arch and its branches and thoracic descending aorta; type 3, descending thoracic aorta, abdominal aorta and/or renal arteries; type 4, abdominal aorta and/or renal arteries; type 5, combined features of types 2b and 4. in addition, involvement of the coronary and pulmonary arteries should be indicated. TAK分型的意义？（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1. 基本概念：LVV定义和分型？2. LVV的评估？3. LVV的治疗？临床研究的评估手段（1）血管炎活动度评估：1994 the BVAS v3/WG, 2001 Disease Extent Index (DEI), 血管炎损害评估：1997 VDI2004 combined damage assessment (CDA)临床研究的评估手段（2）生活质量评估（QOL）：SF-36 EuroQol five-dimensions questionnaire (EQ-5D) no correlations between SF-36 scores and angiographic type, disease duration, ESR or CRP levels in the TAK patients. A negative correlation was observed between the VDI and SF-36 scores临床研究的评估手段（3）TAK-specific disease activity measures1994 NIH2004 DEI-Takayasu (DEI.Tak)2006 Indian Takayasu Clinical Activity Score (ITAS 2010)TAK-specific damage measures临床上怎么评估？imageFDG-PET MRIUSCT1.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，RA 6，AAV 5），44例不详3.156/416：接受治疗4.Positice PET:using authors’ 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NIH scale评估活动度。敏感性 0.86 0.84，特异性0.73 0.84.PET用于TAK活动性评估与其他指标的一致性：2.Significant vascular uptake(moderate and high ) inNo markers of activity vs. 3/3 markers of activity: 8/12 vs. 14/16(67% vs. 88%)P值Visual gradingSUVmax ratioclinical activity0.010.01CRP0.040.0006ESR0.040.02imageFDG-PET MRA: