A Case of Limited Wegener's Granulomatosis Successfully Treated with Methotrexate and Sulfamethoxazole-Trimethoprim and Methyl-Prednisolone

Abstract

A patient with limited Wegener's granulomatosis (WG), who developed nasal involvement before the active generalized symptoms appeared, is reported. A 48 year-old male presented with a complaint of nasal pain resistant to treatment for 3 months. A diagnosis of early stage WG was made based on a positive finding of circulating antineutrophil cytoplasmic antibodies (C-ANCA). The pathognomonic diagnosis of WG had not been confirmed by repeated biopsies of the nasal mucosal lesions. Although a combination of prednisolone plus cyclophosphamide (CY) therapy greatly improves the prognosis in patients with generalized WG, this treatment may be too aggressive for patients with limited WG because of unfavorable drug side effects such as an increased occurrence of infection and cancer of the urinary bladder. In contrast, the risk of toxicity from methotrexate (MTX) appears to be less than CY, and thus MTX was provided instead. Daily systemic treatment with methotrexate and sulfamethoxazole-trimethoprim and methylprednisolone resulted in a marked improvement in the nasal symptoms within weeks. This treatment regimen is an alternative to the conventional and more aggressive immunosuppressive therapy, and may be useful for patients whose illnesses are not immediately life-threatening.