IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies

Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating an...

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Bibliographic Details
Published in:Journal of nephropathology 2024-03, Vol.13 (2), p.e17371-e17371
Main Authors: Alves, Patricia, Góis, Mário, Pereira, Tiago, Carvalho, Dulce, Nolasco, Fernando
Format: Article
Language:English
Subjects:
anca antibody
anti-gbm disease
atypical anti-gbm disease
iga mediated anti-gbm disease
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Description
Summary:Anti-glomerular basement membrane (anti-GBM) disease is an aggressive small vessel vasculitis usually mediated by IgG autoantibodies. We describe the case of a 73-year-old male with rapidly progressive renal failure that was diagnosed with IgA mediated anti-GBM disease associated with circulating anti-neutrophil cytoplasm antibodies (ANCA), where the diagnosis was established on kidney biopsy by detecting linear deposition of IgA along the GBM on immunofluorescence microscopy. Despite an intensive immunosuppressive regimen with plasmapheresis, steroids and oral cyclophosphamide, the disease progressed to end-stage renal failure and the patient was started on hemodialysis.
ISSN:2251-8363
2251-8819
DOI:10.34172/jnp.2023.17371