C3 肾小球病在法属波利尼西亚非常普遍

IF 4.7 Q2 IMMUNOLOGY

Journal of Translational Autoimmunity Pub Date : 2024-10-29 DOI:10.1016/j.jtauto.2024.100254

Nelly Candela , Nicolas Benichou , Mathilde Lefebvre , Lorraine Gueguen , Paula Vieira-Martins , Carine El Sissy , Hervé Sartelet , Pascale Testevuide , Ronan Delaval , Stanislas Faguer

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引用次数: 0

摘要

目的在环境条件和遗传背景相对相同的一组患者中，比较C3肾小球病（C3G）和感染后急性肾小球肾炎（APIGN）的自然病史。结果C3G的发病率为每10万居民中23例。在4/8（50%）例C3G患者中发现了CFI的复发性变异（p.Arg406His），但其致病性仍难以确定。C3G（16 例）和 APIGN（20 例）的发病特征和肾脏结果大致相似，但肾活检时出现驼峰除外。结论C3G在法属波利尼西亚的发病率很高，这表明存在特定的遗传或环境易感因素。对所有C3为主的肾小球肾炎患者都应采用系统诊断工作流程。

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C3 glomerulopathy is highly prevalent in French Polynesia

Objective

To compare the natural history of C3 glomerulopathy (C3G) to acute post-infectious glomerulonephritis (APIGN) in a cohort of patients with a relative homogeneity of environment conditions and genetic background.

Methods

We retrospectively reviewed the characteristics of all patients with biopsy proven C3G or APIGN referred in 2013–2019 to the only renal unit in French Polynesia.

Results

Point prevalence of C3G is ∼23 cases per 100,000 inhabitants. A recurrent variation of CFI (p.Arg406His) was identified at the heterozygous state in 4/8 (50 %) patients with C3G but its pathogenicity remain elusive. Characteristics at presentation and kidney outcomes were roughly similar between C3G (n = 16) and APIGN (n = 20), excepted for the presence of humps on kidney biopsy.

Conclusions

C3G is highly prevalent in French Polynesia suggesting specific genetic or environmental susceptibility factors. Systematic diagnosis workflow should be proposed to all patients with C3 predominant glomerulonephritis.

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来源期刊

Journal of Translational Autoimmunity Medicine-Immunology and Allergy

CiteScore

7.80

自引率

2.60%

发文量

审稿时长

55 days