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

Chauveau, Bertrand ; Gibier, Jean-Baptiste ; Olagne, Jérôme ; Morel, Antoine ; Aydin, Selda ; McAdoo, Stephen P ; Viallet, Nicolas ; Perrochia, Hélène ; Pambrun, Emilie and Royal, Virginie , et al. (2024) In American Journal of Kidney Diseases
Abstract

RATIONALE & 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 ELISA. We characterized a series of patients with atypical anti-GBM disease.

STUDY DESIGN: Case series.

SETTING & PARTICIPANTS: Patients were identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022.

FINDINGS: Among 38 potential cases, 25 were included. 14 (56%) were female and 23 (92%) had hematuria. Median [interquartile range (IQR)] serum creatinine at diagnosis was 150 [102-203] μmol/L and... (More)

RATIONALE & 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 ELISA. We characterized a series of patients with atypical anti-GBM disease.

STUDY DESIGN: Case series.

SETTING & PARTICIPANTS: Patients were identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022.

FINDINGS: Among 38 potential cases, 25 were included. 14 (56%) were female and 23 (92%) had hematuria. Median [interquartile range (IQR)] serum creatinine at diagnosis was 150 [102-203] μmol/L and median [IQR] urine protein to creatinine ratio was 2.4 [1.3-5.2] g/g. 9 (36%) patients 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 nine patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at one 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 to typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes.

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publication status
epub
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American Journal of Kidney Diseases
publisher
Elsevier
external identifiers
  • scopus:85186933694
  • pmid:38171412
ISSN
1523-6838
DOI
10.1053/j.ajkd.2023.11.003
language
English
LU publication?
yes
id
86a2ea46-40d0-49cd-8447-c7402e7b032c
date added to LUP
2024-01-17 08:56:33
date last changed
2024-04-23 18:13:19
@article{86a2ea46-40d0-49cd-8447-c7402e7b032c,
  abstract     = {{<p>RATIONALE &amp; 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 ELISA. We characterized a series of patients with atypical anti-GBM disease.</p><p>STUDY DESIGN: Case series.</p><p>SETTING &amp; PARTICIPANTS: Patients were identified by the French Nephropathology Group as having atypical anti-GBM nephritis between 2003 and 2022.</p><p>FINDINGS: Among 38 potential cases, 25 were included. 14 (56%) were female and 23 (92%) had hematuria. Median [interquartile range (IQR)] serum creatinine at diagnosis was 150 [102-203] μmol/L and median [IQR] urine protein to creatinine ratio was 2.4 [1.3-5.2] g/g. 9 (36%) patients 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 nine patients (38%) who had a monotypic staining pattern tended to be older with less proteinuria and rarely had crescents. Kidney survival rate at one year was 83% and did not appear to be associated with the light chain restriction.</p><p>LIMITATIONS: Retrospective case series with a limited number of biopsies including electron microscopy.</p><p>CONCLUSIONS: Compared to typical anti-GBM disease, atypical anti-GBM nephritis frequently presents with endocapillary or mesangial proliferative glomerulonephritis pattern and appears to have slower disease progression. Further studies are needed to fully characterize its pathophysiology and associated clinical outcomes.</p>}},
  author       = {{Chauveau, Bertrand and Gibier, Jean-Baptiste and Olagne, Jérôme and Morel, Antoine and Aydin, Selda and McAdoo, Stephen P and Viallet, Nicolas and Perrochia, Hélène and Pambrun, Emilie and Royal, Virginie and Demoulin, Nathalie and Kemeny, Jean-Louis and Philipponnet, Carole and Hertig, Alexandre and Boffa, Jean-Jacques and Plaisier, Emmanuelle and Domenger, Camille and Brochériou, Isabelle and Deltombe, Clément and Duong Van Huyen, Jean-Paul and Buob, David and Roufosse, Candice and Hellmark, Thomas and Audard, Vincent and Mihout, Fabrice and Nasr, Samih H and Renaudin, Karine and Moktefi, Anissa and Rabant, Marion}},
  issn         = {{1523-6838}},
  language     = {{eng}},
  month        = {{01}},
  publisher    = {{Elsevier}},
  series       = {{American Journal of Kidney Diseases}},
  title        = {{Atypical Anti-Glomerular Basement Membrane Nephritis : A Case Series From the French Nephropathology Group}},
  url          = {{http://dx.doi.org/10.1053/j.ajkd.2023.11.003}},
  doi          = {{10.1053/j.ajkd.2023.11.003}},
  year         = {{2024}},
}