Candela Nelly, Benichou Nicolas, Lefebvre Mathilde, Gueguen Lorraine, Vieira-Martins Paula, El Sissy Carine, Sartelet Hervé, Testevuide Pascale, Delaval Ronan, Faguer Stanislas
Service de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French Polynesia.
Département de Néphrologie et Transplantation d'organes, Centre de Référence des maladies rénales rares, Centre Hospitalier Universitaire de Toulouse, Toulouse, France.
J Transl Autoimmun. 2024 Oct 29;9:100254. doi: 10.1016/j.jtauto.2024.100254. eCollection 2024 Dec.
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.
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.
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.
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.
在环境条件和遗传背景相对同质的一组患者中，比较C3肾小球病（C3G）与急性感染后肾小球肾炎（APIGN）的自然病史。
我们回顾性分析了2013年至2019年转诊至法属波利尼西亚唯一肾脏科的所有经活检证实为C3G或APIGN患者的特征。
C3G的点患病率约为每10万居民23例。在4/8（50%）的C3G患者中，发现CFI（p.Arg406His）杂合状态存在反复变异，但其致病性仍不明确。C3G组（n = 16）和APIGN组（n = 20）在临床表现和肾脏结局方面大致相似，但肾活检中驼峰的存在情况除外。
C3G在法属波利尼西亚高度流行，提示存在特定的遗传或环境易感因素。应向所有以C3为主的肾小球肾炎患者推荐系统的诊断流程。
