Case Report: Status epilepticus as the initial presentation of antibody-negative Goodpasture's syndrome

摘要

Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage. Serum anti-GBM antibody was negative but the renal biopsy confirmed the diagnosis by showing typical linear IgG along the GBM on immunofluorescent study. He was treated with plasmapheresis and high-dose steroid in combination with oral cyclophosphamide. His renal function normalised after treatment.