Park Y B, Kim J Y, Linton J A, Jung H J, Lee S K, Shin D H
Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
Yonsei Med J. 2001 Feb;42(1):46-54. doi: 10.3349/ymj.2001.42.1.46.
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.
我们回顾了10例韦格纳肉芽肿病患者，特别强调了韦格纳肉芽肿病早期阶段的特征。所有患者均表现出非特异性症状和体征，因此最初未考虑韦格纳肉芽肿病。然而，一半的患者以鼻/鼻窦的临床或放射学疾病作为主要表现问题，鼻活检显示中性粒细胞微脓肿被栅栏状上皮样细胞和排列不规则的巨细胞包围，这是最具特征性的表现。10名患者中有5名在其他器官（特别是肺或肾）受累之前被认为有长期的浅表现象。10名患者中有4名在就诊时ANCA检测呈无反应性。尽管本研究中回顾的病例数量较少，但ANCA无反应率高于其他较大系列研究。鉴于临床病理未被识别的病例最终会发展为预后不良的全面系统性韦格纳肉芽肿病，韦格纳肉芽肿病早期诊断的重要性再怎么强调也不为过。韦格纳肉芽肿病的诊断应基于对活检组织中其特征性组织学变化（特别是血管外病灶）进行全面细致的检查。
