[Goodpasture's syndrome with neurologic involvement and negative ANCA].
Goodpasture's syndrome is a rare autoimmune disorder characterized by rapidly progressive glomerulonephritis (RPGN) and alveolar hemorrhage in the presence of antiglomerular 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.
Pérez-Suárez
G
G
Servicio de Nefrología, Hospital Universitario de Canarias, La Laguna, Tenerife. gpersuarez@gmail.com
Marrero
D
D
Rodríguez
R
R
Delgado
P
P
Cobo
M
M
González-Posada
J M
JM
Hernández
D
D
D058186:Acute Kidney Injury / Q000209:etiology / Q000601:surgery / Q000628:therapy; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000188:drug therapy / Q000276:immunology / Q000601:surgery / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000032:analysis*; D000927:Anticonvulsants / Q000627:therapeutic use; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D006469:Hemoptysis / Q000209:etiology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D010956:Plasmapheresis; D006435:Renal Dialysis; D012640:Seizures / Q000188:drug therapy / Q000209:etiology*; D014635:Valproic Acid / Q000627:therapeutic use; D020293:Vasculitis, Central Nervous System / Q000209:etiology*; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic; D000927:Anticonvulsants; D007166:Immunosuppressive Agents; D014635:Valproic Acid; D003520:Cyclophosphamide; D008775:Methylprednisolone