Jennette J C, Falk R J, Wilkman A S
Department of Pathology and Medicine, University of North Carolina, Chapel Hill, NC 27599-7525, USA.
Ann Acad Med Singap. 1995 Mar;24(2):248-53.
Anti-neutrophil cytoplasmic autoantibodies (ANCA) have specificity for proteins in the cytoplasmic granules of neutrophilic and the lysosomes of monocytes. ANCA occur in a high proportion of patients with Wegener's granulomatosis, microscopic polyangiitis (microscopic polyarteritis), Churg-Strauss syndrome and certain forms of drug-induced vasculitis. ANCA with different specificities from those in patients with vasculitis occur in patients with inflammatory bowel disease and rheumatoid arthritis. ANCA titres correlate to a degree with disease activity and response to treatment. ANCA are a useful diagnostic marker but because of the low prevalence of ANCA-associated diseases, their positive predictive value is good only in patients with signs and symptoms of vasculitis. In vitro data indicate that ANCA can activate cytokine-primed neutrophils and monocytes, causing them to degranulate, release toxic oxygen metabolites, adhere to endothelial cells, and kill endothelial cells. If these events occur in vivo, ANCA may be directly involved in the pathogenesis of vasculitis.
抗中性粒细胞胞浆自身抗体（ANCA）对中性粒细胞胞浆颗粒及单核细胞溶酶体中的蛋白质具有特异性。ANCA在韦格纳肉芽肿、显微镜下多血管炎（显微镜下多动脉炎）、变应性肉芽肿性血管炎及某些药物性血管炎患者中出现的比例较高。炎症性肠病和类风湿关节炎患者体内会出现与血管炎患者不同特异性的ANCA。ANCA滴度在一定程度上与疾病活动度及治疗反应相关。ANCA是一种有用的诊断标志物，但由于ANCA相关疾病的患病率较低，其阳性预测值仅在有血管炎体征和症状的患者中较好。体外数据表明，ANCA可激活细胞因子预激的中性粒细胞和单核细胞，使其脱颗粒、释放有毒氧代谢产物、黏附于内皮细胞并杀伤内皮细胞。如果这些事件在体内发生，ANCA可能直接参与血管炎的发病机制。
