국한성에서 진행된 전신성 Wegener 육아종 1 예

Abstract

Wegener's granulomatosis is a necrotizing and granulomatous vasculitis which involves upper and lower respiratory tract, kidney and skin. The patient who have the protracted Wegener's granulomatosis live a long peroid without major organ imvolvement, In mild cases, the progression is slow and may not need systemic cyclophosphamide treatment. Some cases, however, demonstrate renal involvement and may result in renal failure and death if adequate treatment is not provided. We experienced a case of protracted Wegener's granulomatosis that was a new concept and has not been reported !n Korea. The diagnosis was confirmed by clinical finding and histopathologic features of tiasue biopsy. This case represents a protracted Wegener's granulomatosis with paranasal sinusitis, rhinitis and skin purpura and progress to a gener alized form with kidney involvement after 6years. Treatment with oral cyclophosphamide, steroid and sulfamethoxazole-trimethoprime result improvement of skin, nasal symptom and labratory parameters.