Patricia Alves
* 
, Mário Góis
, Tiago Pereira, Dulce Carvalho, Fernando Nolasco
1 Nephrology Department, Centro Hospitalar Lisboa Central, Lisboa, Portugal
Abstract
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.
Implication for health policy/practice/research/medical education:
This case-report illustrates the diagnosis and treatment of an IgA mediated form of anti-glomerular basement membrane disease, a rare entity with only 13 cases reported in the literature. Furthermore, this is the first and unique case of IgA-mediated anti-GBM associated with positive circulating ANCA, a novel finding of important clinical value, paired with an education value for nephrology trainees and literature review.
Please cite this paper as: Alves P, Góis M, Pereira T, Carvalho D, Nolasco F. IgA mediated anti-glomerular basement membrane disease with associated circulating anti-neutrophil cytoplasm antibodies. J Nephropathol. 2024;13(2):e17371. DOI: 10.34172/jnp.2023.17371.