Yumoto E, Saeki K, Kadota Y
Department of Otolaryngology, School of Medicine, Ehime University, Japan.
Ear Nose Throat J. 1997 Aug;76(8):571-4.
Subglottic stenosis as a complication of Wegener's granulomatosis (WG) is a relatively rare lesion and is difficult to treat surgically once stenosis becomes sufficiently severe to cause inspiratory dyspnea. Thus, it is important to diagnose WG in its early stages to prevent troublesome subglottic stenosis from developing by initiating immunosuppressive therapy. The authors report on a 30-year-old woman suffering from subglottic stenosis of sudden onset due to protracted WG limited to the head and neck region. She had had exudative otitis media for 13 years and saddle nose and nasal crusting for five years. Repeated biopsies of the nasal mucosa and enzyme-linked immunosorbent assays for cytoplasmic patterns of antineutrophil cytoplasmic autoantibody (cANCA) had failed to establish the diagnosis. However, further histologic examination of the nasal mucosa showed vasculitis, and indirect immunofluorescence detected the presence of cANCA. Thus, the diagnosis of WG was confirmed 13 years after the appearance of the initial symptoms in the ear. The patient was given prednisolone (60 mg/day for five days), which greatly relieved the subglottic stenosis. The prednisolone dosage was then tapered to 5 mg/day.
声门下狭窄作为韦格纳肉芽肿病（WG）的一种并发症是相对罕见的病变，一旦狭窄严重到足以引起吸气性呼吸困难，手术治疗就会变得困难。因此，早期诊断WG很重要，以便通过启动免疫抑制治疗来预防麻烦的声门下狭窄的发生。作者报告了一名30岁女性，因局限于头颈部的迁延性WG突然出现声门下狭窄。她患渗出性中耳炎13年，鞍鼻和鼻痂5年。多次鼻黏膜活检及抗中性粒细胞胞浆自身抗体（cANCA）胞浆型的酶联免疫吸附试验均未能确诊。然而，鼻黏膜的进一步组织学检查显示血管炎，间接免疫荧光检测到cANCA的存在。因此，在耳部最初症状出现13年后确诊为WG。给予患者泼尼松龙（60mg/天，共5天），这极大地缓解了声门下狭窄。然后泼尼松龙剂量逐渐减至5mg/天。
