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

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Resumo: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.
Autores principais:Alves, Patrícia
Outros Autores:Góis, Mário; Pereira, Tiago; Carvalho, Dulce; Nolasco, Fernando
Assunto:IgA mediated anti-GBM disease Atypical anti-GBM disease Anti-GBM disease ANCA antibody HCC NEF
Ano:2024
País:Portugal
Tipo de documento:texto
Tipo de acesso:acesso aberto
Instituição associada:Centro Hospitalar de Lisboa Central, EPE (CHLC)
Idioma:inglês
Origem:Repositório do Centro Hospitalar de Lisboa Central, EPE
Descrição
Resumo: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.