内科学教学课件：血管炎病.pptxVIP

血管炎病(Vasculitides)Types of vesselsNames for vasculitidesLarge vessel vasculitis (LVV)Takayasu arteritis (TAK)Giant cell arteritis (GCA)Medium vessel vasculitis (MVV)Polyarteritis nodosa (PAN)Kawasaki disease (KD)Small vessel vasculitis (SVV)Antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis (AAV)Microscopic polyangiitis (MPA)Granulomatosis with polyangiitis (Wegener’s) (GPA)Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA)Immune complex SVVAnti–glomerular basement membrane (anti-GBM) diseaseCryoglobulinemic vasculitis (CV)IgA vasculitis (Henoch-Scho¨nlein) (IgAV)Hypocomplementemic urticarial vasculitis (HUV) (anti-C1q vasculitis)Names for vasculitidesVariable vessel vasculitis (VVV)Behcet’s disease (BD)Cogan’s syndrome (CS)Single-organ vasculitis (SOV)Cutaneous leukocytoclastic angiitisCutaneous arteritisPrimary central nervous system vasculitisIsolated aortitisOthersVasculitis associated with systemic diseaseLupus vasculitisRheumatoid vasculitisSarcoid vasculitisOthersVasculitis associated with probable etiologyHepatitis C virus–associated cryoglobulinemic vasculitisHepatitis B virus–associated vasculitisSyphilis-associated aortitisDrug-associated immune complex vasculitisDrug-associated ANCA-associated vasculitisCancer-associated vasculitisOthers病因和发病机制病因遗传感染发病机制抗中性粒细胞胞浆抗体（ANCA）抗内皮细胞抗体(AECA)免疫复合物病理血管壁各种炎细胞浸润管壁弹力层和平滑肌层受损，形成动脉瘤和血管扩张管壁各层纤维素样增生和内皮细胞增生，造成血管腔狭窄免疫荧光染色较少或没有免疫复合物以及补体沉积诊断临床表现特殊检查ANCA多普勒、CT、MRI血管造影病理病例1患者女，17岁，因“发热2月余”入院。体温最高达39.6 ℃，不规律。无感咽痛、咳嗽、头痛、关节痛，无尿频、尿急、尿痛，无心悸、胸闷。无体重下降。查体浅表淋巴结无肿大，右侧颈前下方压痛，压痛部位有搏动感，双侧脉搏强弱一致。大动脉炎(Takayasu arteritis, TA)累及主动脉及其主要分支年轻起病（平均22岁）女性居多起病隐匿，早期诊断困难病理急性渗出性炎症慢性非特异性炎症肉芽肿血管腔变化狭窄或闭塞增宽或血管瘤临床表现发热年轻女性“单纯”发热鉴别诊断最重要的疾病其他全身表现血管狭窄、器官缺血症状不特异体征繁又多辅助检查眼底检查缺血引起的一系列表现实验室检查炎症指标升高胸片超声血管造影病理诊断病例272岁男性，低热、消瘦3个月，无关节痛、皮疹、脱发、口腔溃疡及肢体感觉、运动异常，曾用多种抗生素治疗无效。追问，似乎感觉轻微头皮麻木、头痛巨细胞动脉炎（giant cell arteritis, GCA）老年起病女性多见容易与风湿性多肌痛并存多以发热来诊老年人“单纯”发热鉴别诊断最重要的疾病因颞动脉受累常见，又称为颞动脉炎GCA病理临床表现全身表现：发热，厌食，消瘦，盗汗颅动脉头痛、头皮压痛视朦间歇性下颌运动障碍(Jaw claudication)