Estenosis subglótica en granulomatosis con poliangitis (granulomatosis de Wegener): presentación de 4 casos / Subglottic stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): report of 4 cases

Introducción. La estenosis subglótica (ESG) en la granulomatosis con poliangitis (GPA) puede ser consecuencia de la enfermedad activa o de procesos inflamatorios repetitivos. Nuestro objetivo es describir las características clínicas y el tratamiento de los pacientes con ESG. Métodos. Estudio descriptivo retrospectivo de los casos diagnosticados durante el período comprendido entre el 1 de enero del 2000 y el 1 de junio del 2015. Resultados. Presentamos 4 casos; la ESG se presentó entre los 2 y 144 meses del diagnóstico de la GPA, los síntomas de presentación fueron disnea de esfuerzo y el estridor laríngeo, 3 desarrollaron ESG en ausencia de actividad sistémica. Dos sujetos con ESG grado i fueron tratados con dilatación traqueal, 2 casos presentaron reestenosis y en 3 casos fue necesario la realización de traqueostomía. Conclusión. La ESG presenta una alta morbilidad. La dilatación endoscópica proporciona alivio sintomático; sin embargo, suelen existir recidivas de la estenosis. La obstrucción grave de la vía aérea a menudo requiere de traqueostomía (AU)

Introduction. Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. Methods. We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. Results. We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. Conclusion. SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy (AU)

Subglottic stenosis in granulomatosis with polyangiitis (Wegener's granulomatosis): Report of 4 cases. / Estenosis subglótica en granulomatosis con poliangitis (granulomatosis de Wegener): presentación de 4 casos.

INTRODUCTION: Subglottic stenosis (SGS) in granulomatosis with polyangiitis (GPA) may result from active disease or from chronic recurrent inflammation. The objective of the study was to describe the clinical features and treatment of patients with subglottic stenosis. METHODS: We retrospectively reviewed the medical records of all patients with SGS due to GPA diagnosed at Rheumatology deparment between January 2000 and June 2015. RESULTS: We present 4 cases of SGS at our department during a period of 15 years. The interval between the presentation of the GPA and SGS varied between 2 and 144 months. The leading symptoms of SGS were dyspnoea on exertion and stridor. Three patients presented SGS without evidence of systemic activity. Two patients presented SGS grade i and received tracheal dilatation; two recurred and three needed a tracheostomy due to severe airway-limiting stenosis. CONCLUSION: SGS presents high morbidity. Even though subglottic dilatation provides symptomatic relief, recurrences may present. Severe airway-limiting stenosis often requires tracheostomy.