Head and Neck Manifestations of Granulomatosis with Polyangiitis: A Retrospective analysis of 19 Patients and Review of the Literature
26 November 2017
10 September 2018
01 March 2019 (eFirst)
Introduction Granulomatosis with Polyangiitis (GPA) is a small vessel vasculitis characterized by a necrositing granulomatous inflammation of the upper and lower respiratory tracts and focal/proliferative glomerulonephritis. In more than 70% of the cases, the presenting symptoms are head and neck manifestations that are often misdiagnosed as infectious or allergic in etiology.
Objective The present study provides an analysis of head and neck manifestations in a series of patients diagnosed with GPA. It also evaluates their medical and surgical treatment and provides a review of the relevant literature.
Methods A retrospective analysis of 19 patients diagnosed with GPA at a public tertiary care hospital between 2006 and 2017 was performed.
Results A total of 19 patients were included in the present study, and 16 of them presented head and neck manifestations. Sinonasal symptoms were the most common, affecting 56% of the patients, followed by laryngotracheal (31.25%) and ear (25%) symptoms. In 7 patients, sinonasal symptoms were the first manifestation of the disease (43.75%). Four patients underwent surgery at some stage of the disease.
Conclusions Head and neck involvement is common in GPA and may stand for the first or the only manifestation of the disease. The otolaryngologists play a central role in the diagnosis and long-term treatment of these patients, and they have to keep this pathology in mind when treating patients with ENT symptoms that do not respond as expected to the treatment.
- 1 Holle JU, Laudien M, Gross WL. Clinical manifestations and treatment of Wegener's granulomatosis. Rheum Dis Clin North Am 2010; 36 (03) 507-526
- 2 Cadoni G, Prelajade D, Campobasso E. , et al. Wegener's granulomatosis: a challenging disease for otorhinolaryngologists. Acta Otolaryngol 2005; 125 (10) 1105-1110
- 3 Cummings CW, Haughey BH, Thomas JR. , et al. Otolaryngology - Head and Neck Surgery. 4th ed. St. Louis, MO: Mosby, Inc; 2005: 934-936 ; 1493–1508.
- 4 Klippel JH. Primer on the Rheumatic Diseases. 12th ed. Atlanta, GA: Arthritis Foundation; 2001: 392-394 , 643.
- 5 Watts RA, Lane SE, Scott DG. , et al. Epidemiology of vasculitis in Europe. Ann Rheum Dis 2001; 60 (12) 1156-1157
- 6 González-Gay MA, García-Porrúa C. Epidemiology of the vasculitides. Rheum Dis Clin North Am 2001; 27 (04) 729-749
- 7 Popa ER, Tervaert JW. The relation between Staphylococcus aureus and Wegener's granulomatosis: current knowledge and future directions. Intern Med 2003; 42 (09) 771-780
- 8 Jennette JC, Xiao H, Falk RJ. Pathogenesis of vascular inflammation by anti-neutrophil cytoplasmic antibodies. J Am Soc Nephrol 2006; 17 (05) 1235-1242
- 9 Erickson VR, Hwang PH. Wegener's granulomatosis: current trends in diagnosis and management. Curr Opin Otolaryngol Head Neck Surg 2007; 15 (03) 170-176
- 10 Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004; 117 (01) 39-50
- 11 Morales-Angulo C, García-Zornoza R, Obeso-Agüera S, Calvo-Alén J, González-Gay MA. [Ear, nose and throat manifestations of Wegener's granulomatosis (granulomatosis with polyangiitis)]. Acta Otorrinolaringol Esp 2012; 63 (03) 206-211
- 12 Srouji IA, Andrews P, Edwards C, Lund VJ. Patterns Granulomatosis. ENT aspects. J Laryngol Otol 2007; 121: 653-659
- 13 Gubbels SP, Barkhuizen A, Hwang PH. Head and neck manifestations of Wegener's granulomatosis. Otolaryngol Clin North Am 2003; 36 (04) 685-705
- 14 D'Anza B, Langford CA, Sindwani R. Sinonasal imaging findings in granulomatosis with polyangiitis (Wegener granulomatosis): A systematic review. Am J Rhinol Allergy 2017; 31 (01) 16-21
- 15 Hoffman GS, Kerr GS, Leavitt RY. , et al. Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 1992; 116 (06) 488-498
- 16 Rasmussen N. Management of the ear, nose, and throat manifestations of Wegener granulomatosis: an otorhinolaryngologist's perspective. Curr Opin Rheumatol 2001; 13 (01) 3-11
- 17 Gluth MB, Shinners PA, Kasperbauer JL. Subglottic stenosis associated with Wegener's granulomatosis. Laryngoscope 2003; 113 (08) 1304-1307
- 18 Congdon D, Sherris DA, Specks U, McDonald T. Long-term follow-up of repair of external nasal deformities in patients with Wegener's granulomatosis. Laryngoscope 2002; 112 (04) 731-737
- 19 Benoudiba F, Marsot-Dupuch K, Rabia MH, Cabanne J, Bobin S, Lasjaunias P. Sinonasal Wegener's granulomatosis: CT characteristics. Neuroradiology 2003; 45 (02) 95-99