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...

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Main Authors: Nelly Candela, Nicolas Benichou, Mathilde Lefebvre, Lorraine Gueguen, Paula Vieira-Martins, Carine El Sissy, Hervé Sartelet, Pascale Testevuide, Ronan Delaval, Stanislas Faguer
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:Journal of Translational Autoimmunity
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Online Access:http://www.sciencedirect.com/science/article/pii/S2589909024000248
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author Nelly Candela
Nicolas Benichou
Mathilde Lefebvre
Lorraine Gueguen
Paula Vieira-Martins
Carine El Sissy
Hervé Sartelet
Pascale Testevuide
Ronan Delaval
Stanislas Faguer
author_facet Nelly Candela
Nicolas Benichou
Mathilde Lefebvre
Lorraine Gueguen
Paula Vieira-Martins
Carine El Sissy
Hervé Sartelet
Pascale Testevuide
Ronan Delaval
Stanislas Faguer
author_sort Nelly Candela
collection DOAJ
description 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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institution Kabale University
issn 2589-9090
language English
publishDate 2024-12-01
publisher Elsevier
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series Journal of Translational Autoimmunity
spelling doaj-art-b3359aaed40943c3bb2be8b773b8ee012024-12-13T11:00:45ZengElsevierJournal of Translational Autoimmunity2589-90902024-12-019100254C3 glomerulopathy is highly prevalent in French PolynesiaNelly Candela0Nicolas Benichou1Mathilde Lefebvre2Lorraine Gueguen3Paula Vieira-Martins4Carine El Sissy5Hervé Sartelet6Pascale Testevuide7Ronan Delaval8Stanislas Faguer9Service 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, FranceService de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French Polynesia; Service de Néphrologie, Groupe Hospitalier Privé Ambroise Paré Hartmann, Neuilly sur Seine, FranceService 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, FranceService de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French PolynesiaLaboratoire d’Immunologie, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, Paris, FranceLaboratoire d’Immunologie, Hôpital Européen Georges Pompidou, Assistance Publique des Hôpitaux de Paris, Paris, FranceLaboratoire d'anatomopathologie, CHU de Nancy, Nancy, FranceService de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French PolynesiaService de Néphrologie, Centre Hospitalier du Taaone, Tahiti, French PolynesiaDépartement de Néphrologie et Transplantation d'organes, Centre de Référence des maladies rénales rares, Centre Hospitalier Universitaire de Toulouse, Toulouse, France; Corresponding author. Department of Nephrology and Organ Transplantation Transplantation, Immunity and Environment (TImE) Research group University Hospital of Toulouse 1, avenue du Pr. Jean Poulhes, 31059, Toulouse Cedex, France.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.http://www.sciencedirect.com/science/article/pii/S2589909024000248C3 glomerulonephritisAcute post-infectious glomerulopathyComplement alternative pathwayComplement factor I
spellingShingle Nelly Candela
Nicolas Benichou
Mathilde Lefebvre
Lorraine Gueguen
Paula Vieira-Martins
Carine El Sissy
Hervé Sartelet
Pascale Testevuide
Ronan Delaval
Stanislas Faguer
C3 glomerulopathy is highly prevalent in French Polynesia
Journal of Translational Autoimmunity
C3 glomerulonephritis
Acute post-infectious glomerulopathy
Complement alternative pathway
Complement factor I
title C3 glomerulopathy is highly prevalent in French Polynesia
title_full C3 glomerulopathy is highly prevalent in French Polynesia
title_fullStr C3 glomerulopathy is highly prevalent in French Polynesia
title_full_unstemmed C3 glomerulopathy is highly prevalent in French Polynesia
title_short C3 glomerulopathy is highly prevalent in French Polynesia
title_sort c3 glomerulopathy is highly prevalent in french polynesia
topic C3 glomerulonephritis
Acute post-infectious glomerulopathy
Complement alternative pathway
Complement factor I
url http://www.sciencedirect.com/science/article/pii/S2589909024000248
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