Jennette J C, Thomas D B, Falk R J
Department of Pathology and Laboratory Medicine, University of North Carolina, Chapel Hill 27599-7525, USA.
Semin Diagn Pathol. 2001 Feb;18(1):3-13.
Microscopic polyangiitis ("microscopic polyarteritis") is a form of necrotizing small vessel vasculitis that most often affects venules, capillaries, arterioles, and small arteries, although it occasionally involves medium-sized arteries. Microscopic polyangiitis is a more appropriate name than microscopic polyarteritis because some patients have no evidence for arterial involvement. The absence or paucity of immunoglobulin localization in vessel walls distinguishes microscopic polyangiitis from immune complex mediated small vessel vasculitis, such as Henoch-Schonlein purpura and cryoglobulinemic vasculitis. Clinical, epidemiological, and pathologic differences warrant the separation of microscopic polyangiitis from polyarteritis nodosa on the basis of involvement of capillaries and venules by the former but not the latter. Pauci-immune necrotizing and crescentic glomerulonephritis, and hemorrhagic pulmonary capillaritis are common in patients with microscopic polyangiitis. Microscopic polyangiitis is the most common cause for pulmonary-renal vasculitic syndrome. The vasculitis in patients with microscopic polyangiitis is pathologically indistinguishable from the vasculitis of Wegener's granulomatosis and Churg-Strauss syndrome. Granulomatous inflammation distinguishes Wegener's granulomatosis from microscopic polyangiitis. Asthma and eosinophilia distinguish Churg-Strauss syndrome from microscopic polyangiitis. Microscopic polyangiitis, Wegener's granulomatosis, and Churg-Strauss syndrome are all associated with circulating antineutrophil cytoplasmic autoantibodies.
显微镜下多血管炎（“显微镜下多动脉炎”）是一种坏死性小血管炎，最常累及小静脉、毛细血管、小动脉和小动脉，不过偶尔也会累及中等大小动脉。显微镜下多血管炎比显微镜下多动脉炎更合适，因为有些患者并无动脉受累的证据。血管壁中免疫球蛋白定位的缺失或稀少将显微镜下多血管炎与免疫复合物介导的小血管炎区分开来，如过敏性紫癜和冷球蛋白血症性血管炎。临床、流行病学及病理差异表明，基于前者累及毛细血管和小静脉而后者不累及，显微镜下多血管炎应与结节性多动脉炎区分开来。寡免疫性坏死性和新月形肾小球肾炎以及出血性肺毛细血管炎在显微镜下多血管炎患者中很常见。显微镜下多血管炎是肺肾血管炎综合征最常见的病因。显微镜下多血管炎患者的血管炎在病理上与韦格纳肉芽肿病和变应性肉芽肿性血管炎的血管炎无法区分。肉芽肿性炎症将韦格纳肉芽肿病与显微镜下多血管炎区分开来。哮喘和嗜酸性粒细胞增多将变应性肉芽肿性血管炎与显微镜下多血管炎区分开来。显微镜下多血管炎、韦格纳肉芽肿病和变应性肉芽肿性血管炎均与循环抗中性粒细胞胞浆自身抗体有关。
