Illum P, Thorling K
Ann Otol Rhinol Laryngol. 1981 May-Jun;90(3 Pt 1):231-5. doi: 10.1177/000348948109000307.
Seventeen patients with Wegener's granulomatosis are presented. The very complex clinical features are outlined. A recently suggested new classification (ELK-classification) is applied. Fourteen patients were treated with cytotoxic agents and steroids, 12 of these receiving 6-mercaptopurine. Of the ten patients still alive, eight are in remission with treatment withdrawn in three cases. The duration of the treatment is discussed. The results are largely satisfactory, but the course of the disease is still capricious. Progression to a higher step in the ELK-classification has been observed in several cases and a number of serious sequelae to the disease are recorded. The initial symptoms of Wegener's granulomatosis are varied and uncharacteristic, and it is important to bear this disease in mind when patients with a long course of apparently trivial infections or peculiar constellations of symptoms from several organs are encountered. Repeated biopsies from the respiratory tract are important in order to establish the diagnosis, but treatment should not be delayed in cases where only a tentative diagnosis can be made on the basis of a reasonably typical clinical picture, even with a negative histological response.
本文报告了17例韦格纳肉芽肿患者。概述了其非常复杂的临床特征。应用了最近提出的一种新分类法（ELK分类法）。14例患者接受了细胞毒性药物和类固醇治疗，其中12例接受了6-巯基嘌呤治疗。在仍存活的10例患者中，8例病情缓解，3例已停止治疗。讨论了治疗持续时间。结果总体上令人满意，但疾病进程仍变幻莫测。在几例病例中观察到病情进展至ELK分类的更高阶段，并记录了该疾病的一些严重后遗症。韦格纳肉芽肿的初始症状多样且无特征性，当遇到有长期看似轻微感染或多个器官出现特殊症状组合的患者时，牢记这种疾病很重要。为了确诊，反复进行呼吸道活检很重要，但在仅根据合理典型的临床表现做出初步诊断（即使组织学反应为阴性）的情况下，治疗不应延迟。
