title: Multilevel airway stenosis in patients with granulomatosis with polyangiitis (Wegener's).
abstract: OBJECTIVE
  To describe the presentation and clinical course of subglottic stenosis (SGS), in particular the development of concurrent airway lesions, in patients with Granulomatosis with Polyangiitis (Wegener's) (GPA).
  
  
  METHODS
  Retrospective review of clinical data from all patients presenting to our institution from 2000 to 2012 with SGS and GPA.
  
  
  RESULTS
  Thirty-five patients were identified. The average age at diagnosis was 33 years old. Eleven patients (31%) presented with SGS as part their initial manifestation of GPA. The remaining patients developed SGS later, at a median of 2.5 years from diagnosis (range 6 months to 14 years). Twelve patients (34%) were noted to have multilevel airway involvement. Seven patients (20%) had documentation of cricoarytenoid joint fixation and vocal cord immobility. This was typically progressive in nature and occurred at an average of two years following the diagnosis of SGS. Six patients (17%) had mid/distal tracheal stenosis and four (11%) had bronchial stenosis. The majority of patients (86%) had evidence of concurrent sinonasal involvement, ten patients (29%) had evidence of otologic involvement and eight (23%) had ocular involvement.
  
  
  CONCLUSIONS
  Cricoarytenoid joint fixation and distal stenosis occur not infrequently in patients with GPA and SGS, resulting in progressive multilevel airway stenosis in about one third of patients. It is critical to identify multilevel stenosis when managing the airways of these patients.
authors: Department of Otorhinolaryngology-Head and Neck Surgery, University of Maryland School of Medicine. Electronic address: eguardiani@smail.umaryland.edu.
authors: Elizabeth
authors: E
authors: Guardiani
authors: Department of Surgery, York Hospital, York, PA.
authors: Hassan Sheikh
authors: HS
authors: Moghaddas
authors: Department of Anesthesiology, Mt. Sinai-Roosevelt Hospital, New York, NY.
authors: Jonathan
authors: J
authors: Lesser
authors: Department of Anesthesiology, Mt. Sinai-Roosevelt Hospital, New York, NY.
authors: Franco
authors: F
authors: Resta-Flarer
authors: Department of Otolaryngology-Head and Neck Surgery, Mt. Sinai-Roosevelt Hospital, New York, NY.
authors: Andrew
authors: A
authors: Blitzer
authors: Department of Surgery, Mt Sinai-Roosevelt Hospital, New York, NY.
authors: Faiz
authors: F
authors: Bhora
authors: Department of Otolaryngology-Head and Neck Surgery, Mt. Sinai-Roosevelt Hospital, New York, NY.
authors: Robert
authors: R
authors: Lebovics
mesh_terms: D000293:Adolescent; D000328:Adult; D000402:Airway Obstruction / Q000453:epidemiology* / Q000473:pathology; D001982:Bronchial Diseases / Q000453:epidemiology* / Q000473:pathology; D002648:Child; D003251:Constriction, Pathologic; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications* / Q000473:pathology*; D006801:Humans; D015994:Incidence; D007829:Laryngostenosis / Q000453:epidemiology* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D013997:Time Factors; D014135:Tracheal Stenosis / Q000453:epidemiology* / Q000473:pathology; D055815:Young Adult