Wegener's Granulomatosis Involving Nasal Cavity / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Wegener's granulomatosis is a chronic multisystem vasculitis. The otolaryngologist has an important role in the early diagnosis of Wegener's granulomatosis because of the high incidence of nasal involvement. In this study, we analyze seven cases of Wegener's granulomatosis involving nasal cavity and describe clinical features, laboratory findings, treatments and outcomes. MATERIALS AND METHOD: Selected for this study were 7 inpatients with Wegener's granulomatosis treated at the department of otolaryngology of Korea University Medical Center from January, 1985 and December, 2002. Their clinicopathologic records were reviewed retrospectively. RESULTS: The nasal manifestations included nasal crusting, nasal obstruction, serous and bloody nasal discharge, septal perforation and saddle nose. All patients showed positive cytoplasmic antineutrophil cytoplasmic antibody (c-ANCA) and an elevated erythrocyte sedimentation rate (ESR). Wegener's granulomatosis was confirmed by the results of nasal mucosal biopsy performed in all patients. CONCLUSION: If multiple and large biopsy sites were selected properly, the diagnostic efficacy of nasal biopsy in Wegener's granulomatosis may increase and it may help early diagnosis since nasal mucosa is the most commonly affected site in Wegener's granulomatosis and nasal cavity is an easily accessible biopsy site.

Clinicopathologic study of Wegener's granulomatosis with special emphasis on early lesions in 10 Korean patients

We reviewed ten cases of Wegener's granulomatosis with special emphasis on the characteristics of the early stage of Wegener's granulomatosis. All patients presented with nonspecific symptoms and signs, so that Wegener's granulomatosis was not initially considered. However, half of the patients had clinical or radiologic disease in the nose/or paranasal sinuses as the primary presenting problems and showed neutrophil microabscess surrounded by palisading epithelioid cells and irregularly arranged giant cells in the nasal biopsy as the most characteristic feature. Five of ten patients were believed to have a protracted superficial phenomenon before involvement of other organs, specifically the lung or kidney. Four of ten patients showed nonreactivity to ANCA test at the time of presentation. Although the number of cases reviewed in this study was small, the rate of nonreactivity to ANCA was higher than those of the larger series. The importance of early diagnosis of Wegener's granulomatosis can not be overemphasized in view of the fact that cases unrecognized clinicopathologically finally progress to full-blown systemic form of Wegener's granulomatosis with poor prognosis. The diagnosis of Wegener's granulomatosis should be based on a thorough and meticulous examination of its characteristic histologic changes in biopsied tissue particularly extravascular foci.