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Park, Kim, Linton, Jung, Lee, and Shin: Clinicopathologic study of Wegener's granulomatosis with special emphasis on early lesions in 10 Korean patients
Original Article
Yonsei Medical Journal

Yonsei Medical Journal 2001; 42(1): 46-54.

Published online: 3 April 2009

DOI: https://doi.org/10.3349/ymj.2001.42.1.46

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

Yong Beom Park1, Jong Yoon Kim1, John A Linton2, Hyeon Joo Jung3, Soo Kon Lee1, Dong Hwan Shin3

1Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.

2Department of Family Medicine, Yonsei University College of Medicine, Seoul, Korea.

3Department of Pathology, Yonsei University College of Medicine, Seoul, Korea.

Dong Hwan Shin, M.D. Department of Pathology, Yonsei University College of Medicine, C.P.O. BOX 8044, Seoul 120-752, Korea, Tel: 82-2-361-5240, Fax: 82-2-362-0860, shindh417@yumc.yonsei.ac.kr

Received 16 September 2000       Accepted 9 November 2000

Copyright © 2001 The Yonsei University College of Medicine

Abstract

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.

Keywords: Wegener's granulomatosis, antineutrophil cytoplasmic antibody, nasal biopsy, early diagnosis, protracted superficial phenomenon

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