The presentation and management of granulomatosis with polyangiitis (Wegener's Granulomatosis) in the pediatric airway.

OBJECTIVE Granulomatosis with polyangiitis (GPA) is a necrotizing inflammatory disease that can affect the airway. The purpose of this study was to present a case of pediatric laryngotracheal GPA and provide management recommendations based on a thorough review of the literature. DATA SOURCES Retrospective chart and literature review REVIEW METHODS: A pediatric patient with laryngotracheal and pulmonary manifestations of GPA who underwent chemotherapy and intralesional corticosteroid injection is described. An extensive literature review of pediatric GPA affecting the larynx/trachea was also performed. RESULTS A pediatric patient presented with acute respiratory distress. Flexible laryngoscopy revealed a laryngeal mass. Magnetic resonance imaging showed circumferential subglottic stenosis, and chest computed tomography demonstrated multiple pulmonary nodules. Laryngeal and tracheal biopsy revealed granulation tissue and primary vasculitis. Labs demonstrated positive cytoplasmic antineutrophil cytoplasmic antibody, consistent with GPA. Methylprednisone, rituximab, cyclophosphamide, and intralesional steroid injection resulted in remission after 12 weeks. Review of the literature revealed two pediatric cases series and 10 case reports of GPA affecting the larynx or trachea. CONCLUSIONS There is a higher prevalence of GPA of the airway in children when compared to adults. Biopsy of the airway lesion may not be necessary and has lower diagnostic yield compared to other GPA subsites. Medical management includes induction therapy followed by maintenance therapy once the disease is in remission. Most patients will require a surgical intervention to maintain the airway. LEVEL OF EVIDENCE NA Laryngoscope, 127:233-240, 2017.