Research by Dr. Rosenbaum is funded in part by Research to Prevent Blindness (New York), the Stan and Madelle Rosenfeld Family Trust, the William and Mary Bauman Foundation, and the William C. Kuzell Foundation. The authors have no other funding, financial relationships, or conflicts of interest to disclose.
Head and Neck
Progression and management of Wegener's granulomatosis in the head and neck†
Article first published online: 5 JUN 2012
Copyright © 2012 The American Laryngological, Rhinological, and Otological Society, Inc.
Volume 122, Issue 8, pages 1695–1700, August 2012
How to Cite
Taylor, S. C., Clayburgh, D. R., Rosenbaum, J. T. and Schindler, J. S. (2012), Progression and management of Wegener's granulomatosis in the head and neck. The Laryngoscope, 122: 1695–1700. doi: 10.1002/lary.23273
- Issue published online: 25 JUL 2012
- Article first published online: 5 JUN 2012
- Manuscript Accepted: 1 FEB 2012
- Manuscript Revised: 19 JAN 2012
- Manuscript Received: 26 DEC 2011
- Granulomatosis with polyangiitis;
- Wegener's granulomatosis;
- head and neck;
- Level of Evidence: 4
To describe the otolaryngologic presentation and natural history of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, and to compare otolaryngologic outcomes of patients with systemic GPA to those with a limited form of GPA confined to the head and neck.
Retrospective chart review.
Review of GPA cases (identified by serology, biopsy, or clinical presentation) seen in the otolaryngology department of an academic medical center.
A total of 24 patients were identified; each patient was followed for an average 6.8 years. Sinusitis and subglottic stenosis were the most commonly observed head and neck manifestations at diagnosis, seen in 64% and 36%, respectively. Over time, disease spread to additional sites in more than half the cohort (n = 14), but only two of 13 patients with disease initially limited to the head and neck developed pulmonary disease, and none developed renal disease. Cumulatively, otitis media was more likely to be observed in patients with systemic disease (P = .04). Patients with localized (n = 12) and systemic (n = 12) GPA did not have significantly different rates of surgical interventions (0.55 vs. 0.72 surgical interventions/patient-year of follow-up, respectively, P = .19).
GPA has a variety of head and neck manifestations, most commonly sinusitis, nasal disease, subglottic stenosis, and otitis media. GPA commonly progresses to involve additional sites, regardless of the extent of disease. These patients require frequent surgical intervention, and the clinician should remain vigilant for progression of disease.