Franssen C F, Stegeman C A, Kallenberg C G, Gans R O, De Jong P E, Hoorntje S J, Tervaert J W
Department of Internal Medicine, Divisions of Nephrology and Clinical Immunology, University Hospital Groningen, Groningen, The Netherlands.
Kidney Int. 2000 Jun;57(6):2195-206. doi: 10.1046/j.1523-1755.2000.00080.x.
Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led some investigators to prefer combining these diseases under the common heading of ANCA-associated vasculitides. However, it is increasingly recognized that there are characteristic differences between patients with anti-PR3 and those with anti-MPO-associated vasculitis. This review focuses on the clinical, histopathologic, and possibly pathophysiologic differences between anti-PR3- and anti-MPO-associated vasculitis. Although there is considerable overlap, the anti-PR3- and anti-MPO-associated vasculitides are each characterized by particular clinical and histopathological findings. Extrarenal organ manifestations and respiratory tract granulomas occur more frequently in patients with anti-PR3 than in those with anti-MPO. Anti-PR3-positive patients with NCGN generally have a more dramatic deterioration of their renal function compared with anti-MPO-positive patients. The term "ANCA-associated vasculitis" is considered as a useful concept in the presence of systemic vasculitis. Likewise, in the presence of vasculitis, the terms "anti-PR3-associated vasculitis" and "anti-MPO-associated vasculitis" are useful concepts.
抗蛋白酶3和抗髓过氧化物酶相关血管炎。韦格纳肉芽肿、显微镜下多血管炎和特发性寡免疫坏死性新月体性肾小球肾炎（NCGN）与针对蛋白酶3（抗PR3）或髓过氧化物酶（抗MPO）的抗中性粒细胞胞浆自身抗体（ANCA）密切相关。这使得一些研究者倾向于将这些疾病合并在ANCA相关血管炎这一共同标题下。然而，越来越多的人认识到抗PR3患者和抗MPO相关血管炎患者之间存在特征性差异。本综述重点关注抗PR3和抗MPO相关血管炎之间的临床、组织病理学以及可能的病理生理学差异。尽管存在相当多的重叠，但抗PR3和抗MPO相关血管炎各自具有特定的临床和组织病理学表现。抗PR3患者的肾外器官表现和呼吸道肉芽肿比抗MPO患者更常见。与抗MPO阳性患者相比，抗PR3阳性的NCGN患者肾功能通常恶化得更明显。在存在系统性血管炎的情况下，“ANCA相关血管炎”这一术语被认为是一个有用的概念。同样，在存在血管炎的情况下，“抗PR3相关血管炎”和“抗MPO相关血管炎”这两个术语也是有用的概念。
