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

 Yong Beom Park  ;  Jong Yoon Kim  ;  John A Linton  ;  Hyeon Joo Jung  ;  Soo Kon Lee  ;  Dong Hwan Shin 
 YONSEI MEDICAL JOURNAL, Vol.42(1) : 46-54, 2001 
Journal Title
Issue Date
Adolescent ; Adult ; Antibodies, Antineutrophil Cytoplasmic/blood ; Female ; Granulomatosis with Polyangiitis/diagnosis ; Granulomatosis with Polyangiitis/drug therapy ; Granulomatosis with Polyangiitis/pathology* ; Humans ; Male ; Middle Aged ; Retrospective Studies
Wegener's granulomatosis ; antineutrophil cytoplasmic antibody ; nasal biopsy ; early diagnosis ; protracted superficial phenomenon
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.
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1. College of Medicine (의과대학) > Dept. of Internal Medicine (내과학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Family Medicine (가정의학교실) > 1. Journal Papers
1. College of Medicine (의과대학) > Dept. of Pathology (병리학교실) > 1. Journal Papers
Yonsei Authors
Kim, Jong Youn(김종윤) ORCID logo https://orcid.org/0000-0001-7040-8771
Park, Yong Beom(박용범)
Shin, Dong Hwan(신동환)
Lee, Soo Kon(이수곤)
Linton, John A.(인요한) ORCID logo https://orcid.org/0000-0001-8000-3049
Jeong, Hyeon Joo(정현주) ORCID logo https://orcid.org/0000-0002-9695-1227
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