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[韦格纳肉芽肿病的临床病程]

[Clinical course of Wegener's granulomatosis].

作者信息

Wurbs W

出版信息

Z Erkr Atmungsorgane. 1983;160(3):253-62.

PMID:6880292
Abstract

Granulomatous necrosing ulcerations in the upper respiratory tract, a generalized arteritis developing lung infarcts, and a focal glomerulonephritis in this sequence characterize the classic progression of Wegener's granulomatosis (WG). Two cases of this rarely observed disease are being reviewed, comparing the typical course of illness with a non-typical form hard to diagnose. On the basis of these cases, considering the available publications, the clinical-roentgenological peculiarities, autoptical findings, and therapeutical possibilities of this usually fatal WG are set forth. Ulcerations of the mucous membrane of the nose and oral cavity or chronic sinusitis, haemoptysis with melted round foci of the lung, which can be roentgenologically proved, as well as increasing pulmonary insufficiency, proteinuria, and haematuria with subsequent renal failure are signs and symptoms of WG. Problems of diagnosis are discussed and difficulties in diagnosing are put forward. Histological confirmation in the early phase of the illness is desirable.

摘要

上呼吸道的肉芽肿性坏死性溃疡、发展为肺梗死的全身性动脉炎以及按此顺序出现的局灶性肾小球肾炎是韦格纳肉芽肿病(WG)的典型病程特征。现回顾两例这种罕见疾病的病例,将典型病程与难以诊断的非典型形式进行比较。基于这些病例,并参考现有文献,阐述了这种通常致命的WG的临床-放射学特点、尸检结果及治疗可能性。鼻腔和口腔黏膜溃疡或慢性鼻窦炎、肺部出现可经放射学证实的圆形融合病灶的咯血、以及逐渐加重的肺功能不全、蛋白尿和血尿并随后发展为肾衰竭,均为WG的体征和症状。文中讨论了诊断问题并提出了诊断难点。在疾病早期进行组织学确诊是可取的。

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