Duncavage J A, Durkin G E, Lehman R H, Toohill R J
Laryngoscope. 1985 Oct;95(10):1178-83. doi: 10.1288/00005537-198510000-00005.
The diagnostic criteria and therapeutic regimens for upper airway necrotizing diseases such as Wegener's granulomatosis, polymorphic reticulosis, and the recently described idiopathic midline destructive disease have been better defined and clarified in the past decades. Despite an improved understanding of the various disease processes, there continues to be difficulty in establishing an early diagnosis before proceeding with prompt treatment to minimize loss of function and cosmetic deformity. To achieve these goals, we have expanded the indications for the use of the cytotoxic drug, cyclophosphamide, in those patients whose clinical and histologic presentations are suggestive of Wegener's granulomatosis, but in whom a specific diagnosis has not been confirmed by biopsy specimen. We have seen ten patients with upper airway lesions of a chronic inflammatory nature over the past six years at the Medical College of Wisconsin affiliated hospitals. Only three of these have had a definitive histopathologic diagnosis of Wegener's granulomatosis made prior to the initiation of cytotoxic therapy. Six have undergone treatment under our expanded criteria without a prior definitive diagnosis. Five of these have had arrest of the disease process, and one has had no response to therapy. One patient was not treated. There have been no serious side-effects from cyclophosphamide therapy in these patients.
在过去几十年中，诸如韦格纳肉芽肿、多形性网状细胞增多症以及最近描述的特发性中线破坏性疾病等上呼吸道坏死性疾病的诊断标准和治疗方案已得到更好的界定和阐明。尽管对各种疾病过程的认识有所提高，但在进行及时治疗以尽量减少功能丧失和美容畸形之前，早期诊断仍存在困难。为实现这些目标，我们扩大了细胞毒性药物环磷酰胺在那些临床和组织学表现提示韦格纳肉芽肿但活检标本尚未确诊的患者中的应用指征。在过去六年里，我们在威斯康星医学院附属医院见过10例具有慢性炎症性质上呼吸道病变的患者。其中只有3例在开始细胞毒性治疗之前就得到了韦格纳肉芽肿的确切组织病理学诊断。6例在我们扩大的标准下接受了治疗，之前没有明确诊断。其中5例病情得到控制，1例对治疗无反应。1例患者未接受治疗。这些患者接受环磷酰胺治疗后未出现严重副作用。
