• Benjamin Terrier, Agnès Dechartres, Charlotte Girard, Stéphane Jouneau, Jean-Emmanuel Kahn, Robin Dhote, Estibaliz Lazaro, Jean Cabane, Thomas Papo, Nicolas Schleinitz, Pascal Cohen, Edouard Begon, Pauline Belenotti, Dominique Chauveau, Elisabeth Diot, Thierry Généreau, Mohamed Hamidou, Gilles Hayem, Guillaume Le Guenno, Véronique Le Guern, Marc Michel, Guillaume Moulis, Xavier Puéchal, Sophie Rivière, Maxime Samson, François Gonin, Claire Le Jeunne, Pascal Corlieu, Luc Mouthon, Loic Guillevin, and French Vasculitis Study Group.
• Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Université Paris Descartes, benjamin.terrier@cch.aphp.fr.
• Rheumatology (Oxford). 2015 Oct 1; 54 (10): 1852-7.

ObjectivesTracheobronchial stenosis (TBS) is noted in 12-23% of patients with granulomatosis with polyangiitis (GPA), and includes subglottic stenosis and bronchial stenosis. We aimed to analyse the endoscopic management of TBS in GPA and to identify factors associated with the efficacy of endoscopic interventions.MethodsWe conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS.ResultsCompared with patients without TBS, those with TBS were younger, more frequently female and had less frequent kidney, ocular and gastrointestinal involvement and mononeuritis multiplex. Endoscopic procedures included 137 tracheal and 50 bronchial interventions, mainly endoscopic dilatation, local steroid injection and conservative laser surgery, and less frequently stenting. After the first endoscopic procedure, the cumulative incidence of endoscopic treatment failure was 49% at 1 year, 70% at 2 years and 80% at 5 years. Factors significantly associated with a higher cumulative incidence of treatment failure were a shorter time from GPA diagnosis to endoscopic procedure [hazard ratio (HR) 1.08 (95% CI 1.01, 1.14); P = 0.01] and a bronchial stenosis [HR 1.96 (95% CI 1.28, 3.00); P = 0.002]. A prednisone dose ≥30 mg/day at the time of the procedure was associated with a lower cumulative incidence of treatment failure [HR 0.53 (95% CI 0.31, 0.89); P = 0.02].ConclusionTBS represents severe and refractory manifestations with a high rate of restenosis. High-dose systemic CSs at the time of the procedure and increased time from GPA diagnosis to bronchoscopic intervention are associated with a better event-free survival. In contrast, bronchial stenoses are associated with a higher rate of restenosis than subglottic stenosis.© The Author 2015. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

39 keywords...

hide…