Halstead L A, Karmody C S, Wolff S M
Otolaryngol Head Neck Surg. 1986 Mar;94(3):368-71. doi: 10.1177/019459988609400320.
We have reviewed 50 cases of Wegener's granulomatosis, seen at the New England Medical Center Hospital between 1970 and 1984, and were impressed that 10 (20%) of these patients were under 25 years of age, with ages ranging from 13 to 23 years. Closer examination of this younger group revealed striking differences in their presenting symptoms and organ involvement when compared to the older group of patients. The presentation of these young patients was varied, with no single predominant symptom. Patients presented with otalgia and otitis media or hearing loss, fulminant sinusitis, arthralgias, and even corneal ulcers. Only one patient had "typical" rhinitis and nasal congestion. This group also had a disproportionate number of patients with involvement of the oral cavity, skin, and trachea. Biopsy of these sites frequently demonstrated necrotizing vasculitis. Three of our 50 patients had intracranial involvement, leading to transient hemiplegia in the first, permanent hemiplegia in the second, and a seizure disorder in the third. Two of these patients were in the younger age group. The proportion of patients with limited and generalized Wegener's granulomatosis was the same in both the younger and older age groups. All the younger patients, however, had manifestations of the disease in the head and neck, while four of the older patients had no symptoms in the upper respiratory tract. The number of young patients in our study emphasizes the fact that Wegener's granulomatosis, indeed, occurs in the younger patient and with a greater frequency than previously supposed. This study suggests that in the teenager and young adult, with an unusual constellation of symptoms of the head and neck and accompanying systemic problems, a diagnosis of Wegener's granulomatosis should be seriously considered.
我们回顾了1970年至1984年间在新英格兰医学中心医院就诊的50例韦格纳肉芽肿病患者，发现其中10例（20%）年龄在25岁以下，年龄范围为13至23岁。对这一年轻患者组进行更仔细的检查发现，与老年患者组相比，他们的症状表现和器官受累情况存在显著差异。这些年轻患者的表现各不相同，没有单一的主要症状。患者表现为耳痛、中耳炎或听力丧失、暴发性鼻窦炎、关节痛，甚至角膜溃疡。只有1例患者有“典型”鼻炎和鼻塞。该组口腔、皮肤和气管受累的患者比例也过高。这些部位的活检经常显示坏死性血管炎。我们的50例患者中有3例有颅内受累，第一例导致短暂性偏瘫，第二例导致永久性偏瘫，第三例导致癫痫发作。其中2例患者属于较年轻的年龄组。年轻和老年患者组中局限性和全身性韦格纳肉芽肿病患者的比例相同。然而，所有年轻患者都有头颈部疾病表现，而4例老年患者上呼吸道没有症状。我们研究中的年轻患者数量强调了这样一个事实，即韦格纳肉芽肿病确实发生在较年轻的患者中，而且发病率比以前认为的要高。这项研究表明，对于青少年和年轻成年人，若出现头颈部症状异常组合并伴有全身问题，应认真考虑韦格纳肉芽肿病的诊断。
