Wegener肉芽腫および限局型Wegener肉芽腫の病態と病理--限局型Wegener肉芽腫を含めたWegener肉芽腫の新しいcriteriaの提案 (内藤儁教授退官記念論文集)
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Wegener's granulomatosis is characterized by a necrotic and granulomatous changes of the upper or lower respiratory tract and glomerulonephritis and disseminated small vessel vasculitis.In the field of otolaryngology, there are not a few cases with lesions in the upper respiratory tract, similar to those in Wegener's granulomatosis, although they do not fully coincide with the definition established by Godman in 1954. These conditions are named as localized Wegener's granulomatosis by Carrington et al.Clinical pictures of 15 cases of generalized or localized Wegener's granulomatosis (8 generalized and 7 localized) were presented.The localized form of the disease spares the kidney. Male to female ratio is 4:11. Age distribution is 16 to 63 years (mean age is 31 years). The most comman symptoms are the necrotic and granulomatous changes of the upper respiratory tract. Histologically, vasculitis is not comman in the lesion but giant cell granuloma is usually seen. The most remarkable laboratory findings are the elevated BSR, positive CRP and RA, increased serum complement level (CH50) and serum fibrinogen level, and increased Ig level and decreased A/G ratio.The polymorphonuclear leukocytes from the patients showed remarkable decrease in their chemotactic activity.The other characteristic clinical findings are follows: the saddle nose, laryngeal stenosis, ulceration of the skin and pulmonary involvement respond to corticostedroi herapy.
- 耳鼻咽喉科臨床学会の論文
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