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[1例以巩膜炎为首发临床表现的局限性韦格纳肉芽肿病]

[A case of scleritis as the initial clinical manifestation of limited Wegener's granulomatosis].

作者信息

Hijikata Naoya, Takayanagi Noboru, Yoneda Koichiro, Sugita Yutaka, Kawabata Yoshinori

机构信息

Department of Respirology, Saitama Cardiovascular and Respirology Center.

出版信息

Nihon Kokyuki Gakkai Zasshi. 2009 Nov;47(11):1025-9.

Abstract

A 63-year-old man who was transferred to our hospital had noticed ocular hyperemia, and 2 months later headache, dry cough, nose bleeds and fever. Chest X-ray film showed bilateral infiltrating shadows. He was given a diagnosis of pneumonia at the previous hospital, where antibiotics were administrated with no effect. High-resolution chest computed tomography (HRCT) on admission revealed consolidations distributed predominantly in the bilateral lower lobes and multiple nodules. Consolidations were also mainly distributed around bronchovascular bundles and at subpleural areas. We suspected Wegener's granulomatosis because of his eye lesion and HRCT findings. Ophthalmologic examination after admission revealed bilateral scleritis. The proteinase 3-antineutrophil cytoplasmic antibody level was 51.4 IU/ml. Thoracoscopic lung biopsy (left S8) showed eccentric granulomatous necrotizing vasculitis, which was consistent with Wegener's granulomatosis. We finally diagnosed this case as limited type Wegener's granulomatosis without renal involvement. We administrated both 60 mg/day prednisolone and 100mg/day cyclophosphamide for initial treatment. After this treatment, he remarkably improved. Scleritis was the initial clinical presentation in this case of Wegener's granulomatosis.

摘要

一名63岁男性被转至我院,最初出现眼部充血,2个月后出现头痛、干咳、鼻出血和发热。胸部X线片显示双侧浸润影。他在前一家医院被诊断为肺炎,接受抗生素治疗无效。入院时的高分辨率胸部计算机断层扫描(HRCT)显示实变主要分布在双侧下叶及多个结节。实变也主要分布在支气管血管束周围和胸膜下区域。由于其眼部病变及HRCT表现,我们怀疑为韦格纳肉芽肿。入院后眼科检查发现双侧巩膜炎。蛋白酶3抗中性粒细胞胞浆抗体水平为51.4 IU/ml。胸腔镜肺活检(左S8)显示偏心性肉芽肿性坏死性血管炎,符合韦格纳肉芽肿。我们最终将该病例诊断为无肾脏受累的局限性韦格纳肉芽肿。初始治疗给予泼尼松龙60mg/天和环磷酰胺100mg/天。治疗后,他明显好转。巩膜炎是该例韦格纳肉芽肿的初始临床表现。

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