Vasculitis of the upper airways

• Published in: Swiss medical weekly Vol. 142; p. w13541
• Main Authors: Pagnoux, Christian, Wolter, Nikolaus E
• Format: Journal Article
• Language: English
• Published: Switzerland 2012
• Subjects: Biopsy; Diagnosis, Differential; Global Health; Glucocorticoids - therapeutic use; Humans; Immunosuppressive Agents - therapeutic use; Incidence; Prognosis; Respiratory Mucosa - pathology; Respiratory Tract Diseases - diagnosis; Respiratory Tract Diseases - drug therapy; Respiratory Tract Diseases - epidemiology; Tomography, X-Ray Computed; Vasculitis - diagnosis; Vasculitis - drug therapy; Vasculitis - epidemiology
• ISSN: 1424-7860; 1424-3997
• DOI: 10.4414/smw.2012.13541

Systemic vasculitides are rare and potentially life-threatening diseases. Granulomatosis with polyangiitis (GPA; formerly Wegener's granulomatosis) and Churg Strauss syndrome (possibly to be renamed eosinophilic granulomatosis with polyangiitis; EGPA) are the 2 chief systemic vasculitides which may involve the upper respiratory tract. Chronic allergic rhinitis and nasal polyposis in EGPA, and recurrent sinusitis and/or otitis in both conditions, are not specific and can thus represent real diagnostic challenges if they are the first manifestations of the disease. Nasal septum perforation, saddle nose deformity and/or subglottic stenosis (SGS), although not totally specific, are more suggestive of GPA. Because upper airway manifestations often tend to be refractory to systemic therapy and/or to linger, local treatment represents a major aspect of management of the condition, especially for patients with SGS.