Dilemmas in the management of chronic nasal and sinus inflammatory diseases of unknown etiology

Abstract

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.

Nasal reconstruction in Wegener's disease

“…There is renal involvement in 46 to 90% of cases with proteinuria, hematuria and leucocyturia [6,10]. Skin involvement, purpura, necrotic palpulae and ulcerations is found in 44 to 77% of cases [6,8,10].…”

“…Chest X-ray is positive in 87% of cases [6]. The biopsies at the level of the nasopharynx, positive in 45% of cases, are more useful than biopsies of the lower respiratory and renal tracts [6,8,10,15]. The biopsies at the level of the nasopharynx, positive in 45% of cases, are more useful than biopsies of the lower respiratory and renal tracts [6,8,10,15].…”

Lupus pernio with Involvement of Nasal Cavity and Maxillary Sinus

“…Sarcoidosis affects the nasal cavity in 1-6% of patients [3] and the paranasal sinuses more rarely [4]. However, less than 1% of patients with sarcoidosis have nasal symptoms as their presenting complaint [5].…”