GRANULOMATOSIS WITH POLYANGIITIS AS A CAUSE OF SUBGLOTTIC STENOSIS

TOPIC: Diffuse Lung Disease TYPE: Medical Student/Resident Case Reports INTRODUCTION: Acquired subglottic stenosis is mostly secondary to trauma, and can also arise in the context of chronic inflammatory diseases1. Granulomatosis with polyangiitis (GPA) a systemic disease characterized by small vessel vasculitis granulomatous lesions respiratory tract. Approximately 25% patients GPA develop stenosis2. CASE PRESENTATION: A 48-years-old female, former-smoker (6 pack-years), rhinosinusitis without surgical history. She was referenced due episodes shortness breath stridor, 2 months evolution. On physical examination, she presented dysphonia saddle nose. Rhinoscopy showed nasal septum destruction mucosa inflammation lots crusts. Cervical-thoracic CT scan revealed extensive sinusopathy concentric thickening, 35mm longitudinal extension 5mm thickness, just below cricoid cartilage resulting reduced airway caliber. Flexible bronchoscopy multiple membranes covering exudative aspects, about 50% reduction tracheal lumen. The patient underwent rigid (RB); bronchial biopsies mechanical dilation were performed. Bronchial compatible nonspecific process. Repeated antineutrophil cytoplasmic antibody (ANCA) tests negative. Three later, experienced clinical worsening emission circumferential membranous rings (molds mucosa); repeated 20% stenosis, immediately distal vocal cords.Limited diagnosed intravenous methylprednisolone pulses done; subsequently maintained immunosuppressive therapy methotrexate prednisolone. evolved recurrence new RB needed, mitomycin-C instillation. Given failure therapy, proposed for rituximab. DISCUSSION: This case illustrates that could be considered localized form GPA, usually ANCA-negative, refractoriness conventional relapsing course3. CONCLUSIONS: Subglottic associated high morbidity. Endoscopic therapies provide symptomatic relief, however frequent. therapeutic approach should customized on case-by-case basis. REFERENCE #1: Rahman, N.A., Fruchter, O., Shitrit, D. et al. bronchoscopic management benign stenosis: long term follow-up 115 patients. J Cardiothorac Surg 5, (2010). https://doi.org/10.1186/1749-8090-5-2 #2: Costantino, C., Niles, J., Wright, Mathisen, D., & Muniappan, A. (2018). Stenosis With Polyangiitis: Role Laryngotracheal Resection. Annals Of Thoracic Surgery, 105(1), 249-253. doi: 10.1016/j.athoracsur.2017.07.026 #3: GARCIA-VALLADARES, I., ESPINOZA, L. (2011). Is Form Limited Wegener's Granulomatosis. Journal Rheumatology, 38(10), 2268-2268. 10.3899/jrheum.110371 DISCLOSURES: No relevant relationships Mariana Argel, source=Web Response Conceicao, Antonio Correia, Ana Rita Ferro, Sónia Isabel Guerra, António Torres,