Upper airway manifestations of granulomatosis with polyangiitis

Upper airway manifestations, particularly sinonasal manifestations, are encountered frequently in granulomatosis with polyangiitis (GPA). Nasal endoscopy often reveals crusting, friable erythematous mucosa, and granulation. Up to 25% of patients may have a "saddle-nose" deformity as cartilage destruction worsens. Treatment is often complicated by loss of mucociliary function and necrosis, leading to refractory symptoms. Culture-directed antibiotics, topical antibiotic and saline irrigations, and occasional debridement of adherent crusts can reduce the frequency of sinonasal exacerbations and improve obstructive symptoms. Surgery should be reserved for patients unresponsive to maximal medical therapy. Saddle-nose reconstruction is possible in highly selected patients and can improve nasal breathing and resolve anosmia. Up to 20% of patients with GPA have subglottic stenosis; patients with respiratory symptoms should undergo laryngoscopy to assess the presence of subglottic narrowing. Although systemic manifestations of GPA are managed by immunosuppressive therapy, most patients with subglottic stenosis of GPA require surgical management (ie, endoscopic dilation, endoscopic or laser excision, surgical resection followed by reconstruction).