Laryngeal Subsite Analysis of Granulomatosis With Polyangiitis (Wegener’s)

Abstract

Wegener’s granulomatosis; Laryngeal manifestations; Subglottic stenosis

Granulomatosi de Wegener; Manifestacions de la laringe; Estenosi subglòtica

Granulomatosis de Wegener; Manifestaciones de la laringe; Estenosis subglótica

Objective To analyze specific intralaryngeal findings associated with granulomatosis with polyangiitis (GPA). Study Design Retrospective chart review. Setting Tertiary referral center. Methods A retrospective chart review was performed on all patients diagnosed with GPA who were evaluated at the laryngology division of Massachusetts Eye and Ear Infirmary between January 2006 and September 2019. Results Forty-four patients (14 male, 30 female) were evaluated for laryngeal pathology. The mean age at onset was 48 years. Nine patients (21%) were identified with only vocal fold disease, 11 (25%) with subglottic disease, and 8 (18%) with disease at the glottis and subglottis (transglottic). The remaining 16 patients (36%) had a normal airway upon examination although they presented with laryngeal symptoms. Patients with glottic disease had statistically significantly lower voice-related quality of life scores than patients with isolated subglottic stenosis. Conclusions Although laryngeal manifestations of GPA is often described as a subglottic disease presenting with respiratory symptoms, subsite analysis show that only 25% of patients had subglottic disease alone, with similar rates of glottic disease alone. Laryngeal subsites have different epithelial mucosa, function, and physiology, and understanding the specific sites of involvement will determine symptoms and enable better analysis of the underlying mechanisms of disease. Glottic disease is associated with a reduction in vocal fold motion and voice changes. Subglottic involvement presents more frequently with airway symptoms. Further research is necessary to better define the specific regions of laryngeal involvement in patients diagnosed with GPA.