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We report a case of generalized Wegener's granulomatosis (WG) successfully treated with high-dose methotrexate (MTX) and predonisolone (PSL). A 35 year-old men had complaints of auditory disturbance and nasal hemorrhage. Diagnosis of WG was made based on positive proteinase-3 anti-neutrophil cytoplasmic antibody (PR3-ANCA), lung nodules, and focal necrotizing glomerulonephritis revealed by renal biopsy. Treatment with cyclophosphamide (CY) and PSL for 3 months was ineffective for the lung nodules. Then, CY was changed to high dose MTX (18mg/week), and his lung lesions improved. In Japan, it is unusual to treat WG with high-dose MTX, but this treatment may be useful in CY-resistant WG.

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