Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis

• Published in: Arthritis and rheumatism 1996-10, Vol.39 (10), p.1754-1760
• Main Authors: Langford, Carol A., Sneller, Michael C., Hallahan, Claire W., Hoffman, Gary S., Kammerer, William A., Talar‐Williams, Cheryl, Fauci, Anthony S., Lebovics, Robert S.
• Format: Article
• Language: English
• Subjects: Adult; Biological and medical sciences; Dilatation; Diseases of the respiratory system; Follow-Up Studies; Glucocorticoids - administration & dosage; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - drug therapy; Granulomatosis with Polyangiitis - surgery; Humans; Intubation, Intratracheal; Laryngostenosis - drug therapy; Laryngostenosis - etiology; Laryngostenosis - surgery; Medical sciences; Radiotherapy. Instrumental treatment. Physiotherapy. Reeducation. Rehabilitation, orthophony, crenotherapy. Diet therapy and various other treatments (general aspects); Retrospective Studies; Tracheostomy
• ISSN: 0004-3591; 1529-0131
• DOI: 10.1002/art.1780391020

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 patients with intratracheal dilation—glucocorticoid injection therapy. Results. 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 43 patients (49%). In 21 patients (49%), SGS began while the patient was receiving systemic immunosuppressive therapy for disease activity involving other sites. Tracheostomy was required in 10 of 18 patients (56%) who were treated with systemic immunosuppressive therapy. In 20 patients 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 frequently unresponsive to systemic immunosuppressive therapy. Intratracheal dilation—injection therapy provides a safe and effective treatment for WG‐associated SGS and, in the absence of major organ disease activity, should be used without concomitant systemic immunosuppressive agents.