Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience.


OBJECTIVES Tracheobronchial 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. METHODS We conducted a French nationwide retrospective study that included 47 patients with GPA-related TBS. RESULTS Compared 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]. CONCLUSION TBS 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.

DOI: 10.1093/rheumatology/kev129

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@article{Terrier2015GranulomatosisWP, title={Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience.}, author={Benjamin Terrier and Agn{\`e}s Dechartres and Charlotte Girard and St{\'e}phane Jouneau and Jean Emmanuel Kahn and Robin Dh{\^o}te and Estibaliz Lazaro and Jean Cabane and Thomas Papo and Nicolas Schleinitz and Pascal Cohen and Edouard B{\'e}gon and Pauline B{\'e}l{\'e}notti and Dominique Chauveau and Elisabeth Diot and Thierry G{\'e}n{\'e}reau and Mohamed Amine Hamidou and Gilles Hayem and Guillaume le Guenno and V{\'e}ronique le Guern and Marc F. Michel and Guillaume Moulis and Xavier Pu{\'e}chal and Sophie Rivi{\'e}re and Maxime Samson and François Gonin and Claire Le Jeunne and Pascal Corlieu and Luc Mouthon and Loic P Guillevin}, journal={Rheumatology}, year={2015}, volume={54 10}, pages={1852-7} }