Department of Biochemistry, Molecular Medicine and Nutrigenomics, Medical University of Varna, Varna, Bulgaria.
Department of Nephrology, Medical University of Sofia, Sofia, Bulgaria; Clinic of Nephrology, University Hospital - "Tsaritza Yoanna - ISUL", Sofia, Bulgaria.
Med Clin (Barc). 2024 Oct 25;163(8):375-382. doi: 10.1016/j.medcli.2024.05.019. Epub 2024 Jul 14.
Lupus nephritis (LN) is a disease marked by autoantibodies against complement components. Autoantibodies against negative complement regulator factor H (anti-FH) are prevalent in aHUS, are associated with deletion of factor H-related protein 1 (FHR1) gene, and have overt functional consequences. They are also observed in C3 glomerulopathies. The frequency and relevance of anti-FH in LN are poorly studied.
The aim of our investigation was to screen for the presence of anti-FH and FHR1 gene deletion in a cohort of LN patients and to evaluate their association with LN activity.
ELISA test and Western blot for detection of anti-FH and FHR1 deletion were used, respectively. Patients' clinical and laboratory parameters regarding anti-FH role were processed by statistical analysis.
Anti-FH were found at low level in a small number of LN patients - 11.7% (7/60) and were not associated with deletion of FHR1. Anti-FH did not correlate with ANA titers, anti-dsDNA, C3/C4 hypocomplementemia, eGFR, proteinuria, or active urinary sediment in LN patients. A weak correlation was found between anti-FH and anti-C3 levels. Anti-FH were linked with endocapillary proliferation and histological activity index. Four anti-FH positive patients had severe to moderate LN as per the BILAG renal score.
Anti-FH autoantibodies are an accessory finding in LN and are more likely to manifest during the active phase of the disease. Due to their low frequency and plasma levels, they do not seem suitable for routine laboratory investigation in patients with LN.
狼疮性肾炎(LN)是一种以补体成分自身抗体为特征的疾病。抗补体因子 H(anti-FH)自身抗体在 aHUS 中很常见,与因子 H 相关蛋白 1(FHR1)基因缺失相关,并具有明显的功能后果。它们也存在于 C3 肾小球疾病中。抗-FH 在 LN 中的频率和相关性研究甚少。
我们的研究目的是在 LN 患者队列中筛查抗-FH 和 FHR1 基因缺失,并评估其与 LN 活动的相关性。
使用 ELISA 检测和 Western blot 分别检测抗-FH 和 FHR1 缺失。对患者的临床和实验室参数进行统计分析,以评估抗-FH 的作用。
在少数 LN 患者中发现了低水平的抗-FH - 11.7%(7/60),并且与 FHR1 缺失无关。抗-FH 与 ANA 滴度、抗 dsDNA、C3/C4 低补体血症、eGFR、蛋白尿或 LN 患者的活动性尿沉渣无关。抗-FH 与抗 C3 水平呈弱相关。抗-FH 与毛细血管内增生和组织学活动指数相关。根据 BILAG 肾脏评分,4 名抗-FH 阳性患者患有严重至中度 LN。
抗-FH 自身抗体是 LN 的一种辅助发现,更可能在疾病的活动期表现出来。由于其频率低和血浆水平低,它们似乎不适合 LN 患者的常规实验室检查。