大动脉炎并不比小血管炎简单ppt课件

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. GCA TAKGCA age 50yrs raceAsianCaucasian gene HLA-B*52 . HLA- B/MICA, HLA- DQB1/HLA- DRB1, FCGR2A/FCGR3A HLA-DRB1*04 type Time to diagnosis delayed Outcome measures BVAS and Vasculitis Damage Index (VDI) ? CRP, ESR, and characteristic symptoms TAK type 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. 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 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 VDI 2004 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 measures 1994 NIH 2004 DEI-Takayasu (DEI.Tak) 2006 Indian Takayasu Clinical Activity Score (ITAS 2010) TAK-specific damage measures 临床上怎么评估？ image FDG-PET MRI US CT 1.分析包括21项项研究，413例LVV，127例GCA，197例TAK 2.Control：299名患者，226例肿肿瘤，31例其他（感染18，RA 6，AAV 5），44例不详详 3.156/416：接受治疗疗 4.Positice PET:using authors criteria PET用于GCA诊诊断：敏感性 0.89，特异性0.98 ，且研究一致性好 PET用于TAK活动动性评评估：敏感性 0.86，特异性0.73 ，且研究一致性差 PET用于TAK活动动性评评估：均用NIH scale评评估活动动 度。 敏感性 0.86 0.84，特异性0.73 0.84. 1. PET用于TAK活动动性评评估与其他指标标的一致性： 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值值Visual gradingSUVmax ratio clinical activity0.010.01 CRP0.040.0006 ESR0.040.02 image FDG-PET MRA: for structure and wall edema Active/inactive: sen 100%,spe 89%; PPV 92%,NPV 100%. （？） MRA reveal wall edema：active 94% vs. inactive 56% ，且与ESR及CRP无关 sustained vessel wall oedema reflects both activity and damage, although vessel wall oedema occurs early in the pre-stenotic inflammatory stage of the disease US: for structure and wall edema；surface of body CTA: for structure （more accurate） no reports on CTA vs.detailed di