title: Vasculitis of the upper airways.
abstract: 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.
authors.affiliation: Division of Rheumatology, Rebecca McDonald Center for Arthritis and Autoimmune Diseases, Mount Sinai Hospital and University Health Network, Toronto, Ontario, Canada. cpagnoux@msn.com
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.forename: Nikolaus E
authors.initials: NE
authors.lastname: Wolter
mesh_terms: D001706:Biopsy; D003937:Diagnosis, Differential; D014943:Global Health; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D015994:Incidence; D011379:Prognosis; D020545:Respiratory Mucosa / Q000473:pathology*; D012140:Respiratory Tract Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology; D014057:Tomography, X-Ray Computed; D014657:Vasculitis / Q000175:diagnosis / Q000188:drug therapy / Q000453:epidemiology
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents