Kimura Rio, Matsuzawa Naoki, Arimura Yoshihiro, Soejima Akinori, Nakabayashi Kimimasa, Yamada Akira
First Department of Internal Medicine, Kyorin University School of Medicine, Tokyo, Japan.
Am J Kidney Dis. 2004 Apr;43(4):e7-10. doi: 10.1053/j.ajkd.2003.12.038.
An 80-year-old woman who had rapidly progressive glomerulonephritis unaccompanied by systemic vasculitis is described. On renal biopsy, she showed necrotizing crescentic glomerulonephritis by light microscopy and pauci-immune glomerular lesions by immunofluorescent study. No dense deposits were present on electronmicroscopic study. On serum examination, indirect immunofluorescent study showed perinuclear pattern antineutrophil cytoplasmic antibody (ANCA), but myeloperoxidase-ANCA and proteinase 3-ANCA were both negative. Her serum reacted only to azurocidin excluding other ANCA antigens: bactericidal permeability-increasing protein, cathepsin G, elastase, lactoferrin, or lysozyme. Serum creatinine level decreased, and C-reactive protein turned negative after steroid therapy. Azurocidin-ANCA also turned negative. It is suggested that azurocidin-ANCA might have been related to the inflammatory process of pauci-immune necrotizing crescentic glomerulonephritis in this patient.
本文描述了一位80岁的女性患者，患有快速进展性肾小球肾炎，但无系统性血管炎。肾活检显示，光镜下为坏死性新月体性肾小球肾炎，免疫荧光检查显示寡免疫性肾小球病变。电镜检查未见致密沉积物。血清检查中，间接免疫荧光研究显示核周型抗中性粒细胞胞浆抗体（ANCA），但髓过氧化物酶-ANCA和蛋白酶3-ANCA均为阴性。她的血清仅与天青杀素发生反应，排除了其他ANCA抗原：杀菌通透性增强蛋白、组织蛋白酶G、弹性蛋白酶、乳铁蛋白或溶菌酶。类固醇治疗后，血清肌酐水平下降，C反应蛋白转阴。天青杀素-ANCA也转阴。提示天青杀素-ANCA可能与该患者寡免疫性坏死性新月体性肾小球肾炎的炎症过程有关。
