C3 glomerulopathy is highly prevalent in French Polynesia

J Transl Autoimmun. 2024 Oct 29:9:100254. doi: 10.1016/j.jtauto.2024.100254. eCollection 2024 Dec.

Abstract

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.

Keywords: Acute post-infectious glomerulopathy; C3 glomerulonephritis; Complement alternative pathway; Complement factor I.