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Wegener肉芽肿是一种多系统受累的疾病 ,其临床特征为呼吸道肉芽肿病变和系统性坏死性血管炎。患者有针对中性粒细胞嗜天青颗粒中蛋白酶 3的自身抗体 ,抗体的滴度与疾病活动程度相关并可预测疾病是否复发 ,此病用免疫抑制疗法特别有效 ,这些证据都支持Wegener肉芽肿是一种自身免疫病。本文着重讨论了抗中性粒细胞胞浆自身抗体在Wegener肉芽肿发病机理中的作用。
Wegener’s granulomatosis is a multi-systemic disease characterized by respiratory granulomatous lesions and systemic necrotizing vasculitis. Patients have autoantibodies directed against neutrophil azure granules in protease 3 and the antibody titers correlate with the degree of disease activity and predict the recurrence of the disease and are particularly effective with immunosuppressive therapy that supports Wegener’s granuloma Is an autoimmune disease. This article focuses on the role of anti-neutrophil cytoplasmic autoantibodies in the pathogenesis of Wegener’s granulomatosis.