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A 44-year-old man presented with intermittent epistaxis, fever, and polyarthralgias of 2 months' duration. For the previous week, he had had puffiness of the face and reduced urinary output. On physical examination, the blood pressure was 180/100 mm Hg; he was pale and had pedal edema. A saddle nose deformity (Panel A) had developed during the previous 4 months. There were no clinical signs of sinus involvement, but computed tomography (CT) showed bilateral mucosal thickening in the maxillary sinuses. Laboratory evaluation revealed the following values: hemoglobin, 7.5 g per deciliter; blood urea nitrogen, 163 mg per deciliter (58 mmol per liter); and creatinine, 10 mg per deciliter (884 µmol per liter). Urinary examination showed dysmorphic red cells and 2+ albuminuria (urinary protein, 1 g per 24-hour period). CT of the chest showed bilateral multiple nodular lesions (Panel B). The test for antineutrophil cytoplasmic antibody was positive for antiproteinase 3, and the test for antinuclear antibody was negative. Renal biopsy showed a pauci-immune crescentic glomerulonephritis. All cultures for organisms, including Mycobacterium tuberculosis, were negative. A diagnosis of Wegener's granulomatosis was made. The patient was treated with intermittent hemodialysis, pulsed intravenous doses of methylprednisolone and cyclophosphamide, and antihypertensive medications. He had a dramatic response to treatment and did not require dialysis at the time of discharge 3 weeks later. At 1-year follow-up, the patient was doing well, with a serum creatinine level of 3 mg per deciliter (265 µmol per liter) without dialysis.
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