Journal of Anaesthesia & Critical Care Case Reports

Vol 7 | Issue 1 | January-April 2021 | Page 14-17 | Diana T. Phan, John Le

Author: Diana T. Phan [1], John Le [1]

[1] Department of Anesthesiology, Olive View-UCLA Medical Center, Sylmar, California, United States of America.

Address of Correspondence
Dr. Diana T. Phan,
Department of Anesthesiology, Olive View-UCLA Medical Center, Sylmar, California, United States of America.
E-mail: Dphan2@dhs.lacounty.gov

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.

References

1. Mansi IA, Opran A, Rosner F. ANCA-associated small-vessel vasculitis. Am Fam Physician 2002;65:1615-20.
2. Schirmer JH, Wright MN, Herrmann K, et al. Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener’s) is a clinically distinct subset of ANCA-associated vasculitis. Arthritis Rheum 2016;68:2953-2963.
3. Blackabey V, Gan RW, Buglass H, et al. Granulomatosis with polyangiitis causing subglottic stenosis: two cases and their management. AME Case Rep 2018;2:17.
4. Mohammad AJ, Mortensen KH, Babar J, et al. Pulmonary involvement in antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis: the influence of ANCA subtype. J Rheumatol 2017;44:1458-1467.
5. Preto C, Silva A, Alves S, et al. Case Report: The diagnosing challenge of a positive ANCA Vasculitis in the Paediatric Age. Hindawi Case Reports in Pediatrics 2017; Article ID 2962794. https://doi.org/10.1155/2017/2962794.
6. Marroquin-Fabian E, Ruiz N, Mena-Zuniga J, et al. Frequency, treatment, evolution, and factors associated with the presence of tracheobronchial stenoses in granulomatosis with polyangiitis. Retrospective analysis of a case series from a single respiratory referral center. Seminars in Arthritis and Rheumatism 2019;48:714-719.
7. Quinn KA, Gelbard A, Sibley C, et al. Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis. Rheumatology 2019;58:2203-2211.
8. Bohm M, Gonzalez Fernandez MI, Ozen S, et al. Clinical features of childhood granulomatosis with polyangiitis (wegener’s granulomatosis). Pediatr Rheumatol Online J 2014;12:18.
9. James KE, Xiao R, Merkel PA, et al. Clinical course and outcomes of childhood-onset granulomatosis with polyangiitis. Clin Exp Rheumatol 2017;35 (Suppl 103): 202-208

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