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儿童IgA血管炎血清补体成分的评估:一项病例对照研究。

Evaluation of Serum Complement Components in Pediatric IgA Vasculitis: A Case-Control Study.

作者信息

Taşkın Raziye Burcu, Aksu Güzide, Şen Sait, Hakverdi Gülden, Dörtkardeşler Burçe Emine, Conkar Tunçay Secil

机构信息

Department of Pediatric Rheumatology, Faculty of Medicine, Health Sciences University Tepecik Training and Research Hospital, Konak, Izmir 35020, Turkey.

Department of Pediatric Rheumatology and Immunology, Faculty of Medicine, Ege University, Bornova, Izmir 35100, Turkey.

出版信息

Children (Basel). 2025 Aug 20;12(8):1090. doi: 10.3390/children12081090.

Abstract

BACKGROUND

IgA vasculitis (IgAV) represents the most frequently seen form of vasculitis among children. Although it often resolves without intervention, renal involvement (IgAV nephritis) poses a risk for long-term complications. Although the lectin and alternative complement pathways are possible causes in its development, dependable serum biomarkers for the early identification of nephritis remain unavailable.

METHODS

In this prospective case-control study, we examined how the serum levels of a membrane attack complex (sC5b-9), complement factor H (CFH), mannose-binding lectin (MBL), and mannose-binding lectin-associated serine protease-1 (MASP-1) relate to renal involvement in IgAV. These complement proteins were measured in children diagnosed with IgAV and compared to levels in healthy controls (HCs) matched for age and sex.

RESULTS

The study cohort comprised 44 IgAV patients with a median age of 8 years and 34 HCs. The CFH levels were reduced significantly in the patient group (median: 357.31 ng/mL; IQR: 228.32) relative to the controls (median: 543.08 ng/mL; IQR: 504.05) (p < 0.001). This decrease was observed irrespective of the presence of nephritis. There were no significant differences in serum sC5b-9, MBL, or MASP-1 levels between the patients and controls. Furthermore, no correlation emerged between these complement components and renal involvement.

CONCLUSION

The data suggest that lower CFH levels may signal systemic dysregulation of the alternative pathway in IgAV. In contrast, the serum levels of sC5b-9, MBL, and MASP-1 appear inadequate as markers for predicting renal involvement. Further research with larger cohorts that includes genetic analyses and examination of kidney tissue is needed to better define the contribution of complement activation in IgAV-related nephritis.

摘要

背景

IgA 血管炎(IgAV)是儿童中最常见的血管炎形式。尽管它通常无需干预即可缓解,但肾脏受累(IgAV 肾炎)会带来长期并发症的风险。虽然凝集素和替代补体途径可能是其发病原因,但仍缺乏用于早期识别肾炎的可靠血清生物标志物。

方法

在这项前瞻性病例对照研究中,我们研究了膜攻击复合物(sC5b - 9)、补体因子 H(CFH)、甘露糖结合凝集素(MBL)和甘露糖结合凝集素相关丝氨酸蛋白酶 - 1(MASP - 1)的血清水平与 IgAV 肾脏受累情况的关系。在诊断为 IgAV 的儿童中测量这些补体蛋白,并与年龄和性别匹配的健康对照(HCs)的水平进行比较。

结果

研究队列包括 44 例中位年龄为 8 岁的 IgAV 患者和 34 例健康对照。与对照组(中位值:543.08 ng/mL;四分位间距:504.05)相比,患者组的 CFH 水平显著降低(中位值:357.31 ng/mL;四分位间距:228.32)(p < 0.001)。无论是否存在肾炎,均观察到这种降低。患者和对照组之间的血清 sC5b - 9、MBL 或 MASP - 1 水平无显著差异。此外,这些补体成分与肾脏受累之间未发现相关性。

结论

数据表明较低的 CFH 水平可能预示 IgAV 中替代途径的全身失调。相比之下,sC5b - 9、MBL 和 MASP - 1 的血清水平似乎不足以作为预测肾脏受累的标志物。需要对更大的队列进行进一步研究,包括基因分析和肾脏组织检查,以更好地确定补体激活在 IgAV 相关肾炎中的作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/86c9/12384529/78899bb1a1f1/children-12-01090-g001.jpg

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