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Published on: December 15, 2011
C3 glomerulopathy is highly prevalent in French Polynesia
Nelly Candela1,2, Nicolas Benichou1,3, Mathilde Lefebvre1,2
1Service de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French Polynesia.
Insights
C3 glomerulopathy (C3G) is highly prevalent in French Polynesia, similar to acute post-infectious glomerulonephritis (APIGN). Further research into genetic and environmental factors is needed to understand C3G susceptibility.
Area of Science:
- Nephrology
- Immunology
- Genetics
Background:
- C3 glomerulopathy (C3G) and acute post-infectious glomerulonephritis (APIGN) are distinct glomerular diseases.
- Understanding their natural history and prevalence is crucial for diagnosis and management.
Purpose of the Study:
- To compare the clinical presentation and outcomes of C3G and APIGN.
- To investigate the prevalence of C3G in a genetically homogeneous population.
Main Methods:
- Retrospective review of biopsy-proven C3G and APIGN cases from 2013-2019 in French Polynesia.
- Analysis of patient characteristics, kidney biopsy findings, and clinical outcomes.
Main Results:
- The point prevalence of C3G was approximately 23 cases per 100,000 inhabitants.
- A specific CFI gene variant (p.Arg406His) was found in 50% of C3G patients, though its role is unclear.
- Clinical presentation and kidney outcomes were similar between C3G and APIGN, with 'humps' on biopsy being a distinguishing feature for APIGN.
Conclusions:
- High C3G prevalence in French Polynesia suggests unique genetic or environmental factors.
- A systematic diagnostic approach for C3-predominant glomerulonephritis is recommended.
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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