Primary systemic vasculitis
Primary systemic vasculitis
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DOI：
10.1016/s0140-6736(97)80118-3
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发表时间：
1997-02-22
期刊：
影响因子：
168.9
通讯作者：
Adu, D
中科院分区：
文献类型：
--
作者：
Savage, COS;Harper, L;Adu, D
血管炎的病理包括血管壁的炎症和坏死。临床表现取决于受累血管的部位、类型和大小，以及相关炎症特征的严重程度。Kussmaul和Maier（1866）首次对动脉炎进行了死后肉眼可见的描述，即增厚的索状动脉，伴有频繁的结节状突起。随着光学显微镜的使用越来越广泛，很明显，小动脉，毛细血管和小静脉也容易发生血管炎。Davson和他的同事们认识到，一些患者表现为高血压和器官梗死，并且炎症主要影响肌性动脉，这种疾病现在被称为经典型结节性多动脉炎。其他患者表现为快速进行性肾炎，并因肾小球和微血管受累而死于肾衰竭，即显微镜下多血管炎-以前称为显微镜下多动脉炎。逐渐地，描述了具有不同于这些多动脉炎变体的特征的患者组。这些疾病包括韦格纳肉芽肿病、变应性肉芽肿综合征、皮肤过敏性血管炎和川崎。沿着这些疾病，还描述了主要影响大血管的血管炎：大动脉炎和颞动脉炎。其他不同的血管炎包括过敏性紫癜和白切特病。血管炎也被公认为其他几种疾病的继发性病理特征，如类风湿性关节炎和系统性红斑狼疮。人们曾多次尝试对血管炎进行分类。1952年，Zeek将坏死性血管炎分为五种亚型。基于临床特征的后续尝试不是很令人满意，因为综合征之间的临床重叠和观察特征可能随时间推移而演变。基于受影响血管大小的分类（有或无肉芽肿）由于血管炎综合征不考虑血管大小的界限而受挫。然而，这种分类形式一直广泛适用（小组）。病因学并不是广泛分类的有用基础，因为很少有明确的因素被阐明，例如，继发于皮肤超敏反应药物反应的血管炎，丙型肝炎引起的冷球蛋白血症性血管炎，乙型肝炎感染后的结节性多动脉炎，或与恶性疾病相关的血管炎。免疫病理机制是一种替代分类系统，包括免疫复合物血管炎，科克伦和同事在20世纪70年代在动物中进行了研究。与结缔组织疾病相关的血管炎
The pathology of vasculitis involves inflammation and necrosis of blood-vessel walls. The clinical expression depends on the site, type, and size of involved vessels, and the severity of the associated inflammatory features. The first post-mortem macroscopic description of arteritis, thickened cord-like arteries with frequent nodular protrusions, is ascribed to Kussmaul and Maier (1 866). As use of light microscopy became increasingly widespread, it became apparent that small arterioles, capillaries, and venules were also susceptible to vasculitis. Davson and colleagues’ recognised that some patients present with hypertension and organ infarction, and the inflammation predominantly affects muscular arteries-a disease now called classic polyarteritis nodosa. Other patients present with a rapidly progressive nephritis and die from renal failure due to glomerular and microvascular involvement, microscopic polyangiitis-previously called microscopic polyarteritis.’Gradually, groups of patients with features differing from these polyarteritis variants were described. These conditions include Wegener’s granulomatosis, Churg-Strauss syndrome, cutaneous hypersensitivity angiitis and Kawasaki disease. Along with these conditions, vasculitides that predominantly affect large vessels were also described: Takayasu’s arteritis and temporal arteritis. Other distinct vasculitides include Henoch-Schonlein purpura and Behqet’s disease. Vasculitis is also well-recognised as a secondary pathological feature of several other diseases such as rheumatoid arthritis and systemic lupus erythematosus. Many attempts have been made to classify the vasculitides. The first, by Zeek in 1952, classified necrotising vasculitis into five subtypes.’Subsequent attempts based on clinical features were not very satisfactory because of clinical overlap between syndromes and the observation that features may evolve over time. Classifications based on the size of the affected vesselwith or without granulomas-are frustrated by the fact that vasculitic syndromes do not respect vessel size boundaries. Nevertheless, this form of classification has been broadly applicable (panel). Aetiology has not been a useful basis for broad classification since few defined factors are recognised-eg, vasculitis secondary to cutaneous hypersensitivity drug reactions, cryoglobulinaemic vasculitis due to hepatitis C, polyarteritis nodosa after hepatitis-B infection, or vasculitis associated with a malignant disorder. Immunopathogenetic mechanisms are an alternative classification system and include immunecomplex vasculitis, which was studied in animals by Cochrane and colleagues in the 1970s.’Vasculitis associated with connective-tissue diseases has similarities