Montecucco C
Istituto di Terapia Medica, IRCCS Policlinico S. Matteo, University of Pavia, Italy.
Monaldi Arch Chest Dis. 1994 Sep;49(4):323-6.
The presence of autoantibodies to neutrophil cytoplasmic proteinase-3, usually reported as c-ANCA, is virtually diagnostic for Wegener's granulomatosis and related conditions. These antibodies are highly associated with active systemic disease, and may be implicated in pathogenesis. In vitro, c-ANCA can induce a respiratory burst and degranulation of cytokine-primed neutrophils, leading to lysis of cultured endothelial cells. This and other putative pathogenic mechanisms are reviewed.
针对中性粒细胞胞浆蛋白酶-3的自身抗体（通常报道为c-ANCA）的存在实际上可诊断韦格纳肉芽肿及相关病症。这些抗体与活动性全身性疾病高度相关，可能参与发病机制。在体外，c-ANCA可诱导细胞因子预处理的中性粒细胞发生呼吸爆发和脱颗粒，导致培养的内皮细胞溶解。本文对这一及其他可能的致病机制进行了综述。
