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Atypical Anti-Glomerular Basement Membrane Nephritis: A Case Series From the French Nephropathology Group

dc.rights.licenseopenen_US
hal.structure.identifierImmunology from Concept and Experiments to Translation = Immunologie Conceptuelle, Expérimentale et Translationnelle [ImmunoConcept]
dc.contributor.authorCHAUVEAU, Bertrand
dc.contributor.authorGIBIER, Jean-Baptiste
dc.contributor.authorOLAGNE, Jerome
dc.contributor.authorMOREL, Antoine
dc.contributor.authorAYDIN, Selda
dc.contributor.authorMCADOO, Stephen
dc.contributor.authorVIALLET, Nicolas
dc.contributor.authorPERROCHIA, Hélène
dc.contributor.authorPAMBRUN, Emilie
dc.contributor.authorROYAL, Virginie
dc.contributor.authorDEMOULIN, Nathalie
dc.contributor.authorKEMENY, Jean-Louis
dc.contributor.authorPHILIPPONNET, Carole
dc.contributor.authorHERTIG, Alexandre
dc.contributor.authorBOFFA, Jean-Jacques
dc.contributor.authorPLAISIER, Emmanuelle
dc.contributor.authorDOMENGER, Camille
dc.contributor.authorBROCHERIOU, Isabelle
dc.contributor.authorDELTOMBE, Clément
dc.contributor.authorDUONG VAN HUYEN, Jean-Paul
dc.contributor.authorBUOB, David
dc.contributor.authorROUFOSSE, Candice
dc.contributor.authorHELLMARK, Thomas
dc.contributor.authorAUDARD, Vincent
dc.contributor.authorMIHOUT, Fabrice
dc.contributor.authorNASR, Samih
dc.contributor.authorRENAUDIN, Karine
dc.contributor.authorMOKTEFI, Anissa
dc.date.accessioned2024-10-14T14:02:53Z
dc.date.available2024-10-14T14:02:53Z
dc.date.issued2024-01
dc.identifier.urihttps://oskar-bordeaux.fr/handle/20.500.12278/202482
dc.description.abstractEnRationale & Objective: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized by a bright linear immunoglobulin staining along the GBM by immunofluorescence without a diffuse crescentic glomerulonephritis nor serum anti-GBM antibodies by conventional enzyme-linked immunosorbent assay (ELISA). We characterized a series of patients with atypical anti-GBM disease. Study Design: Case series. Setting & Participants: Patients identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022. Findings: Among 38 potential cases, 25 were included, of whom 14 (56%) were female and 23 (92%) had hematuria. The median serum creatinine at diagnosis was 150 (IQR, 102-203) μmol/L and median urine protein-creatinine ratio (UPCR) was 2.4 (IQR, 1.3-5.2) g/g. Nine patients (36%) had endocapillary proliferative glomerulonephritis (GN), 4 (16%) had mesangial proliferative GN, 4 (16%) had membranoproliferative GN, 2 (8%) had pure and focal crescentic GN, 1 (4%) had focal segmental glomerulosclerosis, and 5 had glomeruli that were unremarkable on histopathology. Nine patients (36%) had crescents, involving a median of 9% of glomeruli. Bright linear staining for IgG was seen in 22 cases (88%) and for IgA in 3 cases (12%). The 9 patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at 1 year was 83% and did not appear to be associated with the light chain restriction. Limitations: Retrospective case series with a limited number of biopsies including electron microscopy. Conclusions: Compared with typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with an endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have a slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes. Plain-Language Summary: Atypical anti-glomerular basement membrane (GBM) nephritis is characterized histologically by bright linear immunoglobulin staining along the GBM without diffuse crescentic glomerulonephritis or circulating anti-GBM antibodies. We report a case series of 25 atypical cases of anti-GBM nephritis in collaboration with the French Nephropathology Group. Compared with typical anti-GBM disease, we observed a slower disease progression. Patients frequently presented with heavy proteinuria and commonly had evidence of endocapillary or mesangial proliferative glomerulonephritis. About half of the patients displayed a monotypic immune staining pattern; they tended to be older, with less proteinuria, and commonly without glomerular crescents in biopsy specimens. No concomitant circulating monoclonal gammopathy was detected. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes.
dc.language.isoENen_US
dc.subject.enAnti-GBM disease
dc.subject.enAtypical anti-GBM nephritis
dc.subject.enGlomerulonephritis
dc.subject.enKidney biopsy
dc.subject.enRenal pathology
dc.title.enAtypical Anti-Glomerular Basement Membrane Nephritis: A Case Series From the French Nephropathology Group
dc.typeArticle de revueen_US
dc.identifier.doi10.1053/j.ajkd.2023.11.003en_US
dc.subject.halSciences du Vivant [q-bio]/Immunologieen_US
bordeaux.journalAmerican Journal of Kidney Diseasesen_US
bordeaux.page713-728en_US
bordeaux.hal.laboratoriesImmunoConcEpT - UMR 5164en_US
bordeaux.institutionUniversité de Bordeauxen_US
bordeaux.institutionCNRSen_US
bordeaux.peerReviewedouien_US
bordeaux.inpressnonen_US
hal.popularnonen_US
hal.audienceInternationaleen_US
hal.exportfalse
dc.rights.ccPas de Licence CCen_US
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