Ohsawa I, Ohi H, Tamano M, Endo M, Fujita T, Satomura A, Hidaka M, Fuke Y, Matsushita M, Fujita T
Internal Medicine II, Nihon University School of Medicine, Tokyo, Japan.
Clin Immunol. 2001 Oct;101(1):59-66. doi: 10.1006/clim.2001.5098.
Serological and histological studies were carried out to explore the role of the lectin complement pathway in the pathogenesis of cryoglobulinemic glomerulonephritis. Sixteen patients with mixed cryoglobulinemia type II with glomerulonephritis (GN) were enrolled. All cases had hepatitis C virus (HCV) infection. The serum concentration of mannose-binding lectin (MBL) was significantly higher in the GN patients than in the normal controls according to ELISA (P < 0.01). IgG, IgM, C1q, C4d, HCV envelope antigen, MBL, and MBL-associated serine protease-1 (MASP-1) could be visualized in the cryoprecipitate of the 16 patients by Dot blot assay. Renal biopsy specimens obtained from 3 patients were examined by immunohistochemistry, and the glomeruli strongly stained for IgG, IgM, MBL, MASP-1, C4d, C3c, and C3d in a fringe-like pattern. The pattern of HCV constituent deposition was partially fringe-like. The complement profiles of the 16 cases were distinctive; briefly, the serum levels of C1q, C2, and C3 were reduced, although the levels of circulating regulatory proteins (C1-inhibitor, factor H, and factor I) were in the normal range. The serum C4 level was significantly reduced. These results indicate that immune complex formation involves molecules of the lectin pathway and leads to organ damage in cryoglobulinemic glomerulonephritis.
进行了血清学和组织学研究，以探讨凝集素补体途径在冷球蛋白血症性肾小球肾炎发病机制中的作用。纳入了16例患有肾小球肾炎（GN）的II型混合性冷球蛋白血症患者。所有病例均有丙型肝炎病毒（HCV）感染。根据酶联免疫吸附测定（ELISA），GN患者血清中甘露糖结合凝集素（MBL）浓度显著高于正常对照（P<0.01）。通过斑点印迹法可在16例患者的冷沉淀物中检测到IgG、IgM、C1q、C4d、HCV包膜抗原、MBL和MBL相关丝氨酸蛋白酶-1（MASP-1）。对3例患者的肾活检标本进行免疫组织化学检查，肾小球呈边缘样强烈染色，可检测到IgG、IgM、MBL、MASP-1、C4d、C3c和C3d。HCV成分沉积模式部分呈边缘样。16例患者的补体谱具有特征性；简而言之，血清C1q、C2和C3水平降低，尽管循环调节蛋白（C1抑制物、因子H和因子I）水平在正常范围内。血清C4水平显著降低。这些结果表明，免疫复合物形成涉及凝集素途径分子，并导致冷球蛋白血症性肾小球肾炎的器官损伤。
