Gülhan Bora, Orhan Diclehan, Kale Gülsev, Besbas Nesrin, Özen Seza
Department of Pediatric Nephrology, Faculty of Medicine, Hacettepe University, Sıhhiye, Ankara, 06100, Turkey,
Pediatr Nephrol. 2015 Aug;30(8):1269-77. doi: 10.1007/s00467-015-3051-4. Epub 2015 Feb 4.
Immunoglobulin A vasculitis (IgA-V), formerly known as Henoch-Schönlein purpura (HSP), is the most common small vessel vasculitis in children. In recent years, the role of T cells in the pathogenesis of HSP/IgA-V has become a focus of research.
Renal biopsy specimens from 22 pediatric patients diagnosed with Henoch-Schönlein nephritis (patient group) were compared to normal renal tissue in nephrectomy specimens from 20 pediatric patients diagnosed with Wilms tumor (control group). All renal specimens were scored according to International Study of Kidney Disease in Children (ISKDC) and Oxford classification. Immunohistochemical analyses of interferon-gamma (IFN-γ), interleukin (IL)-4, IL-17 and FOXP3 expression were performed.
All glomeruli and tubules of the HSP/IgA-V patients showed significantly higher IFN-γ and IL-17 expression than those of the control group. Glomerular IFN-γ and IL-17 staining grades correlated with the urinary protein/creatinine ratio (r = 0.62, p = 0.02 and r = 0.507, p = 0.016, respectively). IL-17 expression also correlated with the percentage of crescents (r = 0.518, p = 0.014). IL-4 staining was present in only nine of the 22 patient biopsies and did not correlate with any of the parameters studied. Interstitial areas of patient biopsies had more FOXP3+ cells/μm(2) than those of the control group (p < 0.001), but differences in glomerular and tubular FOXP3+ levels (cells/μm(2)) between the two groups were not statistically different. The ISKDC and Oxford scores did not correlate with any parameter studied. However, endocapillary hypercellularity did correlate with IFN-γ expression.
Based on these results, we conclude that IFN-γ and IL-17 contribute to HSP/IgA-V in children.
免疫球蛋白A血管炎(IgA-V),以前称为过敏性紫癜(HSP),是儿童中最常见的小血管血管炎。近年来,T细胞在HSP/IgA-V发病机制中的作用已成为研究热点。
将22例诊断为过敏性紫癜性肾炎的儿科患者的肾活检标本(患者组)与20例诊断为肾母细胞瘤的儿科患者肾切除标本中的正常肾组织(对照组)进行比较。所有肾标本均根据儿童肾脏病国际研究(ISKDC)和牛津分类进行评分。对干扰素-γ(IFN-γ)、白细胞介素(IL)-4、IL-17和FOXP3表达进行免疫组织化学分析。
HSP/IgA-V患者的所有肾小球和肾小管中IFN-γ和IL-17表达均显著高于对照组。肾小球IFN-γ和IL-17染色分级与尿蛋白/肌酐比值相关(分别为r = 0.62,p = 0.02和r = 0.507,p = 0.016)。IL-17表达也与新月体百分比相关(r = 0.518,p = 0.014)。22例患者活检标本中仅有9例存在IL-4染色,且与所研究的任何参数均无相关性。患者活检标本的间质区域中FOXP3+细胞/μm²多于对照组(p < 0.001),但两组之间肾小球和肾小管FOXP3+水平(细胞/μm²)的差异无统计学意义。ISKDC和牛津评分与所研究的任何参数均无相关性。然而,毛细血管内细胞增多确实与IFN-γ表达相关。
基于这些结果,我们得出结论,IFN-γ和IL-17在儿童HSP/IgA-V中起作用。