Abstract
An 18-year-old female was admitted to our ICU for dyspnea. She had bloody sputum, hypoxia and hypoproteinemia with proteinuria. Massive doses of methylprednisolone (750mg·day-1) were administered for 3 days. She recovered temporarily. Renal biopsy revealed crescentic glomerulonephritis and transbronchial lung biopsy revealed alveolar hemorrhage. The examination of autoantibodies was positive for perinuclear antineutrophil cytoplasmic autoantibodies (P-ANCA). Pulmonary and renal function deteriorated gradually despite the administration of prednisolone (60mg·day-1). Plasma exchange and hemodialysis were performed and massive doses of methylprednisolone (1g·day-1) were administered for 3 days. Pulmonary function recovered, but it was necessary to continue hemodialysis. Pulmonary function deteriorated again one month later. P-ANCA tested negative after plasma absorption, but pulmonary function grew worse as abnormalities in blood coagulation progressed. The patient died from hemorrhagic cerebral infarction. We believe this was a case of Wegener's vasculitis with P-ANCA. We administered of massive doses of methylprednisolone, and performed plasma exchange and plasma absorption, but these therapies proved to be ineffective. The levels of P-ANCA, white blood cells and C-reactive protein did not reflect the virulence of the disease in this patient. It is important to investigate the indices that reflect the activity of this disease and the choice of the appropriate therapy for the level of virulence.
