Pediatric ANCA- Associated Vasculitis Presenting with Acute Postoperative Subglottic Stenosis: A Case Report

Abstract

Introduction: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) often present with nonspecific constitutional symptoms. However, there has been increasing recognition of a subgroup of young, female AAV patients who are at higher risk for isolated subglottic airway disease. Case Presentation: We report a rare case of a 17-year-old female with undiagnosed ANCA-associated vasculitis who presents with acute, life threatening subglottic stenosis requiring emergent intubation after routine surgery. Conclusion: Much of the research on ANCA-associated vasculitis have been based on adult cases, however many pediatric patients may not follow the same diagnostic and clinical presentations. Clinicians should be vigilant in recognizing a subgroup of vasculitis patients who are often young, female, and have a higher prevalence of subglottic stenosis. Symptoms should be promptly worked up and initiation of immunosuppressive therapy is necessary to prevent life threatening complications and end-organ damage. Keywords: ANCA-associated vasculitis, Subglottic stenosis, Granulomatosis with polyangiitis.