CLINICAL-FEATURES AND THERAPEUTIC MANAGEMENT OF SUBGLOTTIC STENOSIS IN PATIENTS WITH WEGENERS GRANULOMATOSIS

Objective. To determine the clinical features and optimal treatment of subglottic stenosis (SGS) in patients with Wegener's granulomatosis ( WG). Methods. Review of 43 patients with SGS and treatment of 20 patie nts with intratracheal dilation-glucocorticoid injection therapy. Resu lts. SGS developed in 43 of 189 patients with WG who were followed up at the National Institutes of Health Clinical Center. The diagnosis of SGS occurred in the absence of other features of active WG in 21 of 4 3 patients (49%). In 21 patients (49%), SGS began while the patient wa s receiving systemic immunosuppressive therapy for disease activity in volving other sites. Tracheostomy was required in 10 of 18 patients (5 6%) who were treated with systemic immunosuppressive therapy. In 20 pa tients treated with intratracheal therapy, none required tracheostomy and 6 with previous tracheostomies were decannulated. Conclusion. SGS often occurs independently of other features of active WG and is frequ ently unresponsive to systemic immunosuppressive therapy. Intratrachea l dilation-injection therapy provides a safe and effective treatment f or WG-associated SGS and, in the absence of major organ disease activi ty, should be used without concomitant systemic immunosuppressive agen ts.