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上呼吸道的韦格纳肉芽肿病

Wegener's granulomatosis in the upper respiratory tract.

作者信息

Zidar N, Volavsek M, Trcek C, Kern I, Gale N

机构信息

Institute of Pathology, Faculty of Medicine, Ljubljana, Slovenia.

出版信息

Wien Klin Wochenschr. 2000 Aug 25;112(15-16):676-9.

Abstract

Wegener's granulomatosis is a distinct clinico-pathological entity characterised by necrotising vasculitis of small arteries and veins in conjunction with the formation of granuloma in the upper and lower respiratory tracts, and glomerulonephritis. The vast majority of patients have antineutrophil cytoplasmic antibodies in the serum with a characteristic cytoplasmic pattern. However, in early phases of the disease only the upper respiratory tract may be affected, clinical and histological features may be nonspecific, and antineutrophil cytoplasmic antibodies not present. In this paper we present four patients with involvement of the upper respiratory tract suspicious for early Wegener's granulomatosis. We emphasise the significance of clinical, histological and serological parameters in the early detection of Wegener's granulomatosis.

摘要

韦格纳肉芽肿病是一种独特的临床病理实体,其特征为小动脉和小静脉的坏死性血管炎,同时在上、下呼吸道形成肉芽肿以及肾小球肾炎。绝大多数患者血清中存在抗中性粒细胞胞浆抗体,呈特征性的胞浆型。然而,在疾病早期可能仅上呼吸道受累,临床和组织学特征可能不具特异性,且不存在抗中性粒细胞胞浆抗体。本文报告了4例上呼吸道受累、疑似早期韦格纳肉芽肿病的患者。我们强调临床、组织学和血清学参数在早期诊断韦格纳肉芽肿病中的重要性。

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