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Revista de otorrinolaringología y cirugía de cabeza y cuello

versión On-line ISSN 0718-4816

Resumen

LAGOS V, Antonia; HEIDER C, Claudia; ARAYA C, Macarena  y  MARTIN P, José San. Subglottic stenosis in patients with granulomatosis with polyangiitis. Rev. Otorrinolaringol. Cir. Cabeza Cuello [online]. 2016, vol.76, n.2, pp.184-192. ISSN 0718-4816.  http://dx.doi.org/10.4067/S0718-48162016000200006.

Introduction: Granulomatosis with polyangiitis (GPA) is an autoimmune disease that presents with multiple ENT manifestations. Subglottic stenosis (SGS) has been reported in 8-50% of the patients. Aim: To describe the clinical presentation and treatment of SGS in patients with GPA. Material and method: Retrospective review of clinical data from all patients consulting in the Otolaryngology department at Pontificia Universidad Católica de Chile from 2011 to 2015 with SGS and GPA diagnosis. Results: Ten patients (90% female) with average age at diagnosis of44.6 years. The symptoms were: dyspnea (9/10), dysphonia (6/10) and stridor (5/10). SGS was the initial presenting manifestation of GPA in 80% of patients. GPA was localized in 90% and systemic in 10% of patients, and anti-neutrophilic cytoplasmic antibodies (ANCA) were negative in 60% of patients. Systemic glucocorticoids were used in every patient, associated with immunosuppressants and/or monoclonal antibodies. One patient required tracheostomy and 2 patients were treated with endoscopic balloon dilation associated with local methylprednisolone injection. Conclusions: SGS can be GPA’s initial or unique manifestation. Diagnosis is hindered due to the unspecificity ofsymptoms and the high probability of presenting with negative ANCA. Individualized and multidisciplinary treatment is required. Most patients were treated pharmacologically with an adequate response in our series.

Palabras clave : Subglottic stenosis; granulomatosis with polyangiitis; vasculitis; Wegener granulomatosis.

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