Fibrillary glomerulonephritis and immunotactoid (microtubular) glomerulopathy are associated with distinct immunologic features
Fibrillary glomerulonephritis and immunotactoid (microtubular) glomerulopathy are associated with distinct immunologic features
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DOI：
10.1046/j.1523-1755.2002.00628.x
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发表时间：
2002-11-01
影响因子：
19.6
通讯作者：
Touchard, G
中科院分区：
文献类型：
--
作者：
Bridoux, F;Hugue, V;Touchard, G
背景。在超微结构组织的基础上，对两种非淀粉样组织免疫球蛋白（Ig）沉积物(原纤维性免疫球蛋白（FGN）和免疫球蛋白样（微管性）免疫球蛋白（IT/MTGN）鉴别肾小球肾炎（GN）的临床意义进行了讨论。23例有组织的肾小球Ig沉积根据其纤维状或微管状超微结构分为两组。采用免疫荧光显微镜对肾活检样本进行研究，使用抗光链偶联物（所有病例）和抗igg亚类偶联物（13例患者）。在每一组中，我们研究了临床病理特征，相关的单克隆γ病变（通过免疫电泳和/或免疫印迹检测）或b细胞淋巴细胞增生性疾病，化疗的影响和长期肾脏预后。14例IT/MTGN和9例FGN患者的临床症状[高血压、肾病综合征（NS）和血尿]和亚结构的平均直径相似。在13例IT/MTGN患者中，肾小球（IgG1, 2或3）沉积为单型（kappa， 7例；lambda， 6例）。在8例患者中，肾小球沉积物与相同同型的单克隆Ig相关，在血清（5例）和/或淋巴细胞细胞质中检测到（4例），并在7例患者中与淋巴细胞增生性疾病相关。淋巴细胞中单克隆Ig包涵体的超微结构特征与肾小球微管沉积物相似。相比之下，没有FGN患者出现淋巴浆细胞增生或副蛋白血症。8例肾小球Ig沉积为多克隆，3例均含有IgG4。尽管两组患者的生存期和肾脏生存期没有显著差异，但化疗导致10例IT/MTGN患者的NS缓解，血液学参数也有平行改善。鉴别这些肾病的超微结构是很重要的。有组织的微管单克隆沉积（GOMMID）的GN可能占免疫因子样（微管）GN病例的很大比例。
Background. The clinical relevance of distinguishing two types of glomerulonephritis (GN) with non-amyloid organized immunoglobulin (Ig) deposits-fibrillary GN (FGN) and immunotactoid (microtubular) GN (IT/MTGN)-on the basis of ultrastructural organization, is debated.Methods. Twenty-three patients with organized glomerular Ig deposits were classified into two groups based on the fibrillar or microtubular ultrastructural appearance of the deposits. Kidney biopsy samples were studied by immunofluorescence microscopy, using anti-light chain conjugates (all cases) and anti-IgG subclass conjugates (13 patients). In each group, we studied clinicopathological features, associated monoclonal gammapathy (detected by immunoelectrophoresis and/or immunoblot) or B-cell lymphoproliferative disease, effects of chemotherapy and long-term renal outcome.Results. In 14 IT/MTGN and 9 FGN patients, clinical symptoms [hypertension, nephrotic syndrome (NS) and hematuria] and the mean diameters of the substructures were similar. In 13 IT/MTGN patients, glomerular (IgG1, 2 or 3) deposits were monotypic (kappa, 7 cases; lambda, 6 cases). Glomerular deposits were associated with a monoclonal Ig of the same isotype in eight patients, detected in the serum (5 cases), and/or in the cytoplasm of lymphocytes (4 cases), and with lymphoproliferative disease in seven patients. The ultrastructural features of monoclonal Ig inclusions in lymphocytes were similar to those of glomerular microtubular deposits. In contrast, none of the FGN patients presented lymphoplasmocytic proliferation or paraproteinemia. Glomerular Ig deposits were polyclonal in eight cases and contained IgG4 in all three cases studied. Although patient and renal survival did not differ significantly between the two groups, chemotherapy led to remission of NS in ten IT/MTGN patients, with parallel improvement in hematological parameters.Conclusions. The identification of ultrastructural patterns in these nephropathies is important. GN with organized microtubular monoclonal deposits (GOMMID) probably accounts for a large proportion of immunotactoid (microtubular) GN cases.