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Nefrología (Madrid)
On-line version ISSN 1989-2284Print version ISSN 0211-6995
Abstract
PEREZ-SUAREZ, G. et al. Goodpasture´s syndrome with neurologic involvement and negative ANCA. Nefrología (Madr.) [online]. 2010, vol.30, n.5, pp.584-587. ISSN 1989-2284.
Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of anti-glomerular basement membrane (anti-GBM) antibodies. Central nervous system involvement is highly unusual in the absence of anti-neutrophil cytoplasmic antibodies. We report the case of a 20-year-old man with RPGN accompanied by bloody sputum, tonic-clonic seizure and high titers of anti-GBM antibody. After treatment with immunosuppressants and plasmapheresis, the patient showed reduced anti-GBM antibody titers and improved neurologic and respiratory symptoms, but renal failure persisted, requiring hemodialysis. Twenty months later, with the disease in remission, he underwent deceased-donor renal transplantation.
Keywords : Acute renal failure; Goodpastures syndrome; Cerebral vasculitis; Anti-glomerular basement membrane disease.