AB0662 Subglottic stenosis in granulomatosis with poliangiitis

Background The characteristic lesion of laryngotracheal involvement in granulomatosis with polyangiitis (GPA) is subglottic stenosis (SGS), due to active disease or from chronic recurrent inflammation, 8% to 23% during the course of GPA and as initial manifestation in 1% to 6% of cases. Objectives Describe clinical features and treatment of patients with SGS Methods We retrospectively reviewed the medical records of 46 patients with SGS due to GPA diagnosed at a Rheumatology department (2000–2017). We retrospectively reviewed the medical records of 46 patients with SGS due to GPA diagnosed at a Rheumatology department (2000–2017). Results 6/46 patients with GPA diagnosis presented SGS (13%). 83% women, mean age of 37.8 years (DS ±14.8). Mean time between the presentation of the GPA and the diagnosis of SGS:18 months. Most frequent clinical manifestations of SGS: dyspnea (83%), stridor (83%), dysphonia (50%), cough (33%), bronchospasm (16%). One patient presented SGS as initial manifestation of the disease. Mean BVAS:14. Two patients presented such complication with evidence of systemic manifestations. Re stenosis was observed in one patient. Treatment: IV CYC 83%, oral CYC 16%, methylprednisolone (MP) 83%, oral steroids, plasma exchange (16%) in each one. Endoscopic intervention and balloon dilatation33%, and intralesional Mitomycin 16%. Conclusions 13% patients with GPA presented SGS, being in the majority of cases associated with other manifestations of the disease. However, 67%had no signs of systemic involvement (localised disease). Local treatment was necessary in only 33% because there was a good response to IS in 67% of the cases. Reference [1] G. Horta-Baas, et al. Subglottic stenosis in granulomatosis with polyangiitis (Wegener’s granulomatosis): Report of 4 cases.Reumatol Clin. 2016;12(5):267–273. (Surgical interventions and local therapy for Wegener’s granulomato) Disclosure of Interest None declared