狼疮性肾炎患者体内的抗因子 H 自身抗体。

IF 2.6 4区 医学 Q1 MEDICINE, GENERAL & INTERNAL Medicina Clinica Pub Date : 2024-10-25 DOI:10.1016/j.medcli.2024.05.019
Galya Mihaylova , Vasil Vasilev , Mariya Kosturkova , Mariana Petkova , Maria Radanova
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引用次数: 0

摘要

导言狼疮性肾炎(LN)是一种以针对补体成分的自身抗体为特征的疾病。针对阴性补体调节因子H(抗-FH)的自身抗体在aHUS中很普遍,与因子H相关蛋白1(FHR1)基因缺失有关,并具有明显的功能性后果。在 C3 肾小球疾病中也可观察到抗 HFH。目的:我们调查的目的是在一组 LN 患者中筛查抗 FH 和 FHR1 基因缺失的存在,并评估它们与 LN 活动的关联:方法:分别采用酶联免疫吸附试验(ELISA)和免疫印迹法(Western blot)检测抗FH基因和FHR1基因缺失。方法:分别采用 ELISA 试验和 Western blot 检测抗-FH 和 FHR1 缺失,并对患者有关抗-FH 作用的临床和实验室参数进行统计分析:少数 LN 患者(11.7%,7/60)抗 FH 水平较低,且与 FHR1 基因缺失无关。抗 FH 与 LN 患者的 ANA 滴度、抗dsDNA、C3/C4 低补体血症、eGFR、蛋白尿或活动性尿沉渣无关。抗 FH 与抗 C3 水平之间存在微弱的相关性。抗 FH 与毛细血管内膜增生和组织学活动指数有关。根据BILAG肾脏评分,4名抗FH阳性患者患有重度至中度LN:抗FH自身抗体是LN的附属发现,更有可能在疾病的活动期表现出来。由于抗FH自身抗体的出现频率和血浆水平较低,它们似乎并不适合用于LN患者的常规实验室检查。
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Anti-factor H autoantibodies in patients with lupus nephritis

Introduction

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.

Aim

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.

Method

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.

Results

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.

Conclusions

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.
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来源期刊
Medicina Clinica
Medicina Clinica 医学-医学:内科
CiteScore
3.10
自引率
5.10%
发文量
295
审稿时长
22 days
期刊介绍: Medicina Clínica, fundada en 1943, es una publicación quincenal dedicada a la promoción de la investigación y de la práctica clínica entre los especialistas de la medicina interna, así como otras especialidades. Son características fundamentales de esta publicación el rigor científico y metodológico de sus artículos, la actualidad de los temas y, sobre todo, su sentido práctico, buscando siempre que la información sea de la mayor utilidad en la práctica clínica.
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