Csernok E, Schmitt W H, Ernst M, Bainton D F, Gross W L
Dept. of Rheumatology, Medical University of Lübeck, Germany.
Adv Exp Med Biol. 1993;336:45-50. doi: 10.1007/978-1-4757-9182-2_5.
We studied the presence of proteinase 3 (PR3), myeloperoxidase (MPO) and elastase (HLE) on the plasma membrane of neutrophils in patients with biopsy-proven Wegener's disease (WG), pANCA-positive vasculitis, control patients (SLE, rheumatoid arthritis, ankylosing spondylitis), sepsis patients and healthy donors. We found an overexpression of PR3 on the cell surface of neutrophils in WG, ANCA-associated vasculitis and during infection (sepsis). Thus PR3 becomes accessible to ANCA. Furthermore we detected intracytoplasmic IgG antibodies in PMN from patients with WG by immunoelectron microscopy and direct immunofluorescence. Our findings support the pathophysiological role of ANCA.
我们研究了经活检证实的韦格纳肉芽肿病（WG）、抗中性粒细胞胞浆抗体（pANCA）阳性血管炎患者、对照患者（系统性红斑狼疮、类风湿关节炎、强直性脊柱炎）、脓毒症患者及健康供者中性粒细胞质膜上蛋白酶3（PR3）、髓过氧化物酶（MPO）和弹性蛋白酶（HLE）的存在情况。我们发现PR3在WG、抗中性粒细胞胞浆抗体（ANCA）相关血管炎患者的中性粒细胞细胞表面以及感染（脓毒症）期间过度表达。因此，PR3可被ANCA识别。此外，我们通过免疫电子显微镜和直接免疫荧光检测到WG患者中性粒细胞胞浆内的IgG抗体。我们的研究结果支持了ANCA的病理生理作用。
