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 pat ients presented with nonspecific symptoms and signs, so that Wegener's gran ulomatosis was not initially considered. However, half of the patients had clinical or radiologic disease in the nose/or paranasal sinuses as the prim ary presenting problems and showed neutrophil microabscess surrounded by pa lisading epithelioid cells and irregularly arranged giant cells in the nasa l biopsy as the most characteristic feature. Five of ten patients were believed to have a protracted superficial phenome non before involvement of other organs, specifically the lung or kidney. Fo ur of ten patients showed nonreactivity to ANCA test at the time of present ation. Although the number of cases reviewed in this study was small, the r ate of nonreactivity to ANCA was higher than those of the larger series. The importance of early diagnosis of Wegener's granulomatosis can not be ov eremphasized in view of the fact that cases unrecognized clinicopathologica lly finally progress to full-blown systemic form of Wegener's granulomatosi s with poor prognosis. The diagnosis of Wegener's granulomatosis should be based on a thorough and meticulous examination of its characteristic histol ogic changes in biopsied tissue particularly extravascular foci.