AB0662 Subglottic stenosis in granulomatosis with poliangiitis. (12th June 2018)

• Record Type: Journal Article
• Authors: Pena, C.E.; Giordano, F.; Garcia, L.; Costi, C.; Pendon, G.; Vulcano, A.; Pereira, D.; Garcia, M.
• Is Part Of: Annals of the rheumatic diseases. Volume 77(2018)Supplement 2
• Page Start: 1475
• Page End: 1475
• Publication Date: 2018-06-12
• Subjects: Rheumatism -- Periodicals; 616.723005
• DOI: 10.1136/annrheumdis-2018-eular.3977
• Languages: English
• ISSNs: 0003-4967

Abstract

Abstract : 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