[Two cases of anti-neutrophil cytoplasmic antibody-negative generalized Wegener's granulomatosis with alveolar hemorrhage]

Anti-neutrophil cytoplasmic antibody (ANCA) is a distinct marker for Wegener's granulomatosis. We report two cases of ANCA-negative Wegener's granulomatosis. A 70-year-old woman (case 1) and a 27-year-old woman (case 2), who had sinus symptoms and renal disorders, were admitted to our hospital with alveolar hemorrhage. Wegener's granulomatosis was diagnosed on biopsy of the nasal mucosa. Case 1 responded well to the combination therapy with cyclophosphamide and prednisolone, while case 2 responded well to pulse therapy with methyl-prednisolone. These findings suggest that patients with ANCA-negative Wegener's granulomatosis may respond well to chemotherapy. This might lead to an improved prognosis in even severe cases, even though the prognosis of generalized Wegener's granulomatosis with alveolar hemorrhage is usually poor.