Abstract
A 65-year-old woman was admitted on November 17, 1989, because of oliguria and generalized edema. She had been suffering from cough and low-grade fever for about 5 months and had taten medications since being diagnosed with pneumonia at another hospital. On admission, physical examination revealed facial pallor, a blood pressure of 180/110mmHg; hematocrit, 25.2%; hemoglobin, 8.2g/dl; platelets, 10.6×104 per μl; serum creatinine, 13.1mg/dl; BUN, 91.9mg/dl; β2MG, 2, 970μg/, l; Fe, 174μg/dl. Urinalysis showed 3+ proteinuria, and 3+ white blood cells and 2+ red blood cells per high-power field. On November 20 and 21, the patient underwent 3 hours of hemodialysis, with nafamostat mesilate instead of heparin, and received blood transfusions. The level of antiglomerular basement-membrane antibody (anti-GBM antibody) was also examined. On November 21 hemoptysis occurred, and a chest roentgenogram revealed diffuse nodular alveolar infiltrates bilaterally. On November 22, there was a sudden increase in hemoptysis and dyspnea, and respiratory distress with sudden fatal cardiopulmonary failure occurred.
The post-mortem anti-GBM antibody was reported to be positive. We reviewed 14 cases of Goodpasture's syndrome reported in Japan since 1981. Hemodialysis was the main therapy in these cases. However, about 50% of patients treated with hemodialysis died. These reports also indicate that pulse therapy or semipulse therapy may be effective.
