Department of Pathology, Faculty of Medicine, Fukuoka University, Nanakuma 7-45-1, Jonan-ku, Fukuoka City, 814-0180, Japan.
Pediatr Nephrol. 2010 Jun;25(6):1091-9. doi: 10.1007/s00467-009-1439-8. Epub 2010 Feb 12.
The aim of our study was to clarify the association between immunoglobulin G(IgG) subclasses and the complement pathway in patients with idiopathic membranous nephropathy (MN). Immunofluorescence (IF) was performed in 16 MN patients and 20 controls using antibodies against IgG, IgA, IgM, C1q, C3c, C4d, IgG1, IgG2, IgG3, IgG4, mannose binding lectin (MBL), C4-binding protein (C4-bp), factor B, C5b-9, and CD59. MN was classified into two types, segmental MN (S-MN; six patients) and global MN (G-MN; ten patients), according to the distribution of IgG deposits along the glomerular capillary wall. No deposition of any antibody was found in the controls. IF revealed IgG1, IgG3, C1q, C3c, C4d, C4-bp, C5b-9, and CD59 deposits in patients with S-MN, whereas IgG1, IgG2, IgG3, IgG4, C3c, C4d, MBL, factor B, C4-bp, C5b-9, and CD59 deposits were detected in those with G-MN. There was a higher deposition of IG1, IgG2, and IgG4 in patients with G-MN than in those with S-MN, whereas the intensity of C1q deposits was higher in S-MN than in G-MN patients. In contrast, the intensity of factor B and MBL was higher in G-MN than in S-MN patients. This is the first report of S-MN patients showing complement activation of the classical pathway associated with IgG1 and IgG3 and G-MN patients showing complement activation of both the alternative and lectin pathways associated with IgG2 and IgG4.
本研究旨在阐明特发性膜性肾病(MN)患者 IgG 亚型与补体途径之间的关系。采用针对 IgG、IgA、IgM、C1q、C3c、C4d、IgG1、IgG2、IgG3、IgG4、甘露聚糖结合凝集素(MBL)、C4 结合蛋白(C4-bp)、因子 B、C5b-9 和 CD59 的抗体,对 16 例 MN 患者和 20 例对照进行免疫荧光(IF)。根据 IgG 沉积在肾小球毛细血管壁上的分布,将 MN 分为节段性 MN(S-MN;6 例)和全球性 MN(G-MN;10 例)。对照中未发现任何抗体沉积。IF 显示 S-MN 患者存在 IgG1、IgG3、C1q、C3c、C4d、C4-bp、C5b-9 和 CD59 沉积,而 G-MN 患者则存在 IgG1、IgG2、IgG3、IgG4、C3c、C4d、MBL、因子 B、C4-bp、C5b-9 和 CD59 沉积。与 S-MN 患者相比,G-MN 患者的 IgG1、IgG2 和 IgG4 沉积更高,而 S-MN 患者的 C1q 沉积强度更高。相反,G-MN 患者的因子 B 和 MBL 沉积强度高于 S-MN 患者。这是首次报道 S-MN 患者表现为与 IgG1 和 IgG3 相关的经典途径补体激活,而 G-MN 患者表现为与 IgG2 和 IgG4 相关的替代和凝集素途径补体激活。