Nasal manifestations of wegener's granulomatosis
Nasal manifestations of wegener's granulomatosis
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韦格纳肉芽肿病的鼻部表现
DOI：
10.1288/00005537-197412000-00002
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发表时间：
1974
期刊：
影响因子：
--
通讯作者：
E. G. Harrison
中科院分区：
文献类型：
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作者：
T. J. Mcdonald;R. Deremee;E. Kern;E. G. Harrison
本文回顾了52例韦格纳肉芽肿的临床表现，强调了耳鼻喉科医师在早期诊断中的作用。所有病例均符合目前公认的条件标准：在具有典型临床病程的患者中存在原发性坏死性血管炎。52例患者中，31例患者有鼻部病变。典型表现为持续性鼻塞，中年患者无鼻部疾病史。在早期，鼻内检查可能仅显示弥漫性鼻粘膜肿胀，无组织破坏。全身症状为不适、盗汗、间歇性发热和游走性关节痛;异常高的沉降率也很常见。随后，典型的鼻表现是粘膜的弥漫性破坏和恶臭的结痂。硬壳下面的组织非常脆弱。鼻中隔穿孔也很常见。对代表性组织进行适当的活检是重要的。这种情况是可以治疗的;目前，正在使用糖皮质激素联合或不联合环磷酰胺。在52名患者中，31名存活; 10名死亡，11名患者的当前信息不可用。
A review of 52 recent cases of Wegener's granulomatosis has clarified the clinical manifestations and has emphasized the otolaryngologist's role in its early diagnosis. All the cases satisfied the currently accepted criteria for the condition: the presence of primary necrotizing vasculitis in a patient with a typical clinical course. Among the 52 patients, 31 patients had nasal lesions. The typical manifestation was persistent nasal obstruction in a middle‐aged patient with no history of nasal disorder. In the early stages, intranasal examination may reveal only diffuse nasal mucosal swelling, without tissue destruction. Systemic symptoms are malaise, night sweats, intermittent pyrexia, and migratory arthralgias; an abnormally high sedimentation rate is also frequent. Later, the typical nasal findings are diffuse destruction of the mucosa and foul‐smelling crusts. The tissue underlying the crusts is extremely friable. Perforations of the nasal septum are also common. Adequate biopsy of representative tissue is important. The condition is treatable; currently, corticosteroids with or without cyclophosphamide are being used. Of the 52 patients, 31 are alive; 10 are dead, and current information is unavailable on 11 patients.