Abbott F, Jones S, Lockwood C M, Rees A J
Department of Medicine, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK.
Nephrol Dial Transplant. 1989;4(1):1-8.
Patients with Wegener's granulomatosis have autoantibodies to a neutrophil cytoplasmic antigen. As these patients often present with severe glomerulonephritis we investigated whether the same autoantigen was expressed in glomeruli by using both cultured human glomerular cells and frozen normal human kidney sections. Glomeruli were isolated from normal human kidney cortex by differential sieving and plated on to coverslips in growth medium containing various supplements. Cell types were identified by a series of markers and designated either epithelial, endothelial or mesangial. In single and double staining experiments, glomerular cells were incubated with a monoclonal antibody to the neutrophil cytoplasmic antigen (MCAW8), control mouse monoclonal IgG, sera from patients with Wegener's granulomatosis and control normal human serum. MCAW8 and Wegener's patient sera bound specifically to cultured epithelial and endothelial cells. MCAW8 was also found to bind to the glomerular epithelium in frozen sections. We conclude that autoantibodies to glomerular antigens are present in serum of patients with Wegener's granulomatosis and may be important in the pathogenesis of rapidly progressive glomerulonephritis.
韦格纳肉芽肿病患者存在针对中性粒细胞胞浆抗原的自身抗体。由于这些患者常表现为严重的肾小球肾炎,我们通过使用培养的人肾小球细胞和冷冻的正常人肾组织切片,研究了肾小球中是否表达相同的自身抗原。通过差速筛分从正常人肾皮质中分离出肾小球,并接种到含有各种补充剂的生长培养基中的盖玻片上。通过一系列标志物鉴定细胞类型,并将其指定为上皮细胞、内皮细胞或系膜细胞。在单染和双染实验中,将肾小球细胞与抗中性粒细胞胞浆抗原的单克隆抗体(MCAW8)、对照小鼠单克隆IgG、韦格纳肉芽肿病患者的血清以及对照正常人血清一起孵育。MCAW8和韦格纳病患者的血清特异性结合培养的上皮细胞和内皮细胞。还发现MCAW8与冷冻切片中的肾小球上皮细胞结合。我们得出结论,韦格纳肉芽肿病患者血清中存在针对肾小球抗原的自身抗体,这可能在快速进行性肾小球肾炎的发病机制中起重要作用。