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肾病综合征患儿肾脏免疫细胞浸润的特征。

Characterisation of renal immune cell infiltrates in children with nephrotic syndrome.

机构信息

Klinik für Kinder und Jugendliche, Friedrich-Alexander-Universität Erlangen-Nürnberg, Loschgestrasse 15, 91054 Erlangen, Germany.

出版信息

Pediatr Nephrol. 2010 Jul;25(7):1291-8. doi: 10.1007/s00467-010-1507-0. Epub 2010 Apr 13.

Abstract

There is increasing evidence that not only T cells but also B cells may play an important role in the pathogenesis of idiopathic nephrotic syndrome (NS). We have evaluated the infiltrating immune cells found in renal biopsies from 38 children with NS using immunohistochemistry techniques involving antibodies against T cells (CD3, CD4, CD8, FoxP3), B cells (CD20), macrophages (CD68) and follicular dendritic cells (CD21). Kidney biopsies with thin basement membrane disease were used as controls. We found higher numbers of interstitial CD3-positive T cells and macrophages in patients with focal segmental glomerulosclerosis (FSGS) than in those with minimal change glomerulopathy (MCGN) and in the controls, and significantly lower FoxP3-positive cells in patients with FSGS, MCGN and steroid-dependent NS than in the controls. Significantly higher numbers of glomerular B cells were found in FSGN patients than in MCGN patients and controls. Of note, in three patients who were later successfully treated with anti-CD20 antibody rituximab, the number of renal B cells was negligible in the preceding biopsy. In conclusion, the higher numbers of interstitial CD3-positive T cells in renal biopsies of pediatric patients with FSGS argue for a higher inflammatory activity. The significantly higher number of glomerular B cells in FSGS patients may indicate a particular pathogenetic role or epiphenomenon in this disease. However, patients with no interstitial or glomerular B cells could also benefit from rituximab treatment.

摘要

越来越多的证据表明,不仅 T 细胞,B 细胞也可能在特发性肾病综合征(NS)的发病机制中发挥重要作用。我们采用免疫组织化学技术,用针对 T 细胞(CD3、CD4、CD8、FoxP3)、B 细胞(CD20)、巨噬细胞(CD68)和滤泡树突状细胞(CD21)的抗体,评估了 38 例 NS 患儿肾活检中浸润的免疫细胞。将薄基底膜疾病的肾活检作为对照。我们发现局灶节段性肾小球硬化(FSGS)患者的间质 CD3 阳性 T 细胞和巨噬细胞数量高于微小病变性肾小球病(MCGN)和对照组,FSGS、MCGN 和激素依赖型 NS 患者的 FoxP3 阳性细胞显著低于对照组。FSGS 患者的肾小球 B 细胞数量明显高于 MCGN 患者和对照组。值得注意的是,在随后用抗 CD20 抗体利妥昔单抗成功治疗的 3 例患者中,前一次肾活检中肾 B 细胞数量可忽略不计。总之,FSGS 患儿肾活检中间质 CD3 阳性 T 细胞数量较多,提示炎症活性较高。FSGS 患者肾小球 B 细胞数量明显增加,可能表明在该疾病中具有特定的致病作用或表现为伴随现象。然而,无间质或肾小球 B 细胞的患者也可能从利妥昔单抗治疗中获益。

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