Esnault V L, Audrain M A, Sesboüé R
Service de Néphrologie-Immunologie clinique, CHU Hôtel-Dieu, Nantes, France.
Exp Clin Immunogenet. 1997;14(3):206-13.
The vasculitic lesions observed in Wegener's granulomatosis may be partly the consequence of proteases released following activation of neutrophils by ANCA. The activity of these proteases, including proteinase 3 (PR3) and elastase, is normally closely restricted to the inflammation site by a large excess of circulating alpha-1-antitrypsin (alpha1AT). Patients with ANCA-positive systemic vasculitis may exhibit a protease/antiprotease imbalance either genetically determined in the rare patients with deficient alpha1AT phenotypes, or more often acquired through both alpha1AT inactivation in various pathological conditions and possible inhibition of PR3/alpha1AT complexation by anti-PR3 ANCA. This imbalance may at least contribute to disease spreading or aggravation.
韦格纳肉芽肿中观察到的血管炎性病变可能部分是抗中性粒细胞胞浆抗体（ANCA）激活中性粒细胞后释放的蛋白酶所致。这些蛋白酶的活性，包括蛋白酶3（PR3）和弹性蛋白酶，通常通过大量循环的α1抗胰蛋白酶（α1AT）紧密限制在炎症部位。ANCA阳性系统性血管炎患者可能表现出蛋白酶/抗蛋白酶失衡，这在罕见的α1AT表型缺陷患者中是由基因决定的，或者更常见的是通过各种病理条件下的α1AT失活以及抗PR3 ANCA对PR3/α1AT复合物形成的可能抑制而获得的。这种失衡可能至少促成疾病的扩散或加重。
