Clinical manifestations and treatment of idiopathic and Wegener granulomatosis - Associated subglottic stenosis

Objective: To compare and contrast the manifestations and surgical management of subglottic stenosis in patients with airway obstruction attributed to granulomatosis with polyangiitis (GPA), previously known as Wegener granulomatosis, and those with idiopathic subglottic stenosis (iSGS). Design: Retrospective medical chart review. Review of subglottic stenosis cases seen in the otolaryngology department of an academic medical center from 2005 through 2010. Data were obtained on disease presentation, operative management. and findings. Setting: Tertiary referral center. Participants: A total of 24 patients with iSGS and 15 patients with GPA-associated subglottic stenosis (GPA-SGS). Results: All individuals with iSGS were female, and 40% of patients with GPA-SGS were male (P<.01). Patients with iSGS tended to have a higher Myer-Cotton stenosis grade at the time of dilation than those with GPA-SGS (P =.02). Individuals with GPA-SGS were more likely to undergo tracheotomy as a result of disease-related complications than individuals with iSGS (P<.01). No patients with an open airway reconstruction in the iSGS group required follow-up mechanical dilation. In contrast, all patients with open airway reconstructions in the GPA-SGS group underwent more than 1 subsequent airway dilation (P<.01). Conclusions: While surgical utilization is the mainstay of treatment in iSGS and GPA-SGS, iSGS occurs almost exclusively in females and presents with a greater degree of stenosis at the time of endoscopic dilation. In contrast, GPA-SGS is associated with greater rates of tracheotomy. Open airway reconstruction may be used in the treatment of iSGS and GPA-SGS but is much more effective in iSGS.