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IgA Mediated Anti-Glomerular Basement Membrane Disease with Associated Circulating Anti-Neutrophil Cytoplasm Antibodies

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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.
Main Authors:Alves, Patrícia
Other Authors:Góis, Mário; Pereira, Tiago; Carvalho, Dulce; Nolasco, Fernando
Subject:IgA mediated anti-GBM disease Atypical anti-GBM disease Anti-GBM disease ANCA antibody HCC NEF
Year:2024
Country:Portugal
Document type:text
Access type:open access
Associated institution:Centro Hospitalar de Lisboa Central, EPE (CHLC)
Language:English
Origin:Repositório do Centro Hospitalar de Lisboa Central, EPE

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