系统性血管炎2013课件.pptVIP

系统性血管炎;Friedrich Wegener，1930;肉芽肿; New Engl J Med. 2005;352:392 Arthritis Rheum. 2008;58:834. ;Lung;早期阶段以中性粒细胞浸润、坏死为突出表现的微脓肿形成 之后多核巨细胞浸润形成非典型肉芽肿性炎症 上述两种改变不是绝对的;Friedrich Wegener，1907—1990 ;2007年：美国胸科医师学会委员会投票一致同意收回授予韦格纳的临床大师奖，并建议更名 2009年：《CHEST》发表专文提出用“坏死性肉芽肿性血管炎（NGV）”的新命名取代WG，但未获广泛认同;2011年：;肉芽肿性多血管炎（Granulomatosis With Polyangiitis，GPA）：Wegener’s Granulomatosis;;microscopic polyarteritis（MPA）改为microscopic polyangiitis ;系统性血管炎（SV）的重新命名 SV的治疗策略变化：诱导缓解，巩固维持 生物制剂的使用;;Large Vessel Vasculitis (LVV): Takayasu Arteritis (TAK) and Giant Cell Arteritis (GCA) Medium Vessel Vasculitis (MVV): Polyarteritis Nodosa (PAN) and Kawasaki Disease (KD) Small Vessel Vasculitis (SVV): ANCA-Associated Vasculitis (AAV) including: Microscopic Polyangiitis (MPA), Granulomatosis with Polyangiitis (Wegener’s) (GPA) and Eosinophilic Granulomatosis with Polyangiitis (Churg Strauss) (EGPA) Immune Complex SVV including: Anti-GBM Disease, Cryoglobulinemic Vasculitis, IgA Vasculitis (Henoch-Sch?nlein) (IgAV) and Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis) (HUV). ;Variable Vessel Vasculitis (VVV): Beh?ets Disease (BD) and Cogan’s Syndrome (CS). Single Organ Vasculitis (SOV): Cutaneous Leukocytoclastic Angiitis, Cutaneous Arteritis, Primary CNS Vasculitis and Isolated Aortitis. Vasculitis Associated with Systemic Disease: Lupus Vasculitis, Rheumatoid Vasculitis and Sarcoid Vasculitis. Vasculitis Associated with Probable Etiology: Hepatitis C Virus-Associated Cryoglobulinemic Vasculitis, Hepatitis B Virus-Associated Vasculitis, Syphilis-Associated Aortitis, Serum Sickness-Associated Immune Complex Vasculitis, Drug-Associated Immune Complex Vasculitis, Drug-Associated ANCA-Associated Vasculitis and Cancer-Associated Vasculitis. ;Chapel Hill 2012年新分类;较为全面的分类 逐渐去除荣誉性命名 缺陷之一：将来要重视影像学检查的新技术，尤其对中大血管炎的诊断和分类以及活动性判断的评估;;女31岁，低热伴咳嗽4月。 辅查：ANA、抗dsDNA、抗ENA、ANCA、RF-，ESR＞100，CRP 60，各项感染、肿瘤筛查-;女57岁，发热伴关节肿痛2月 辅查：ANA、抗dsDNA、抗ENA、ANCA、RF-，ESR＞140，CRP 100，各项感染、肿瘤筛查-;应用 FDG PET-CT 评估LVV的疾病活动性;第2