title: Granulomatosis with polyangiitis: endoscopic management of tracheobronchial stenosis: results from a multicentre experience.
abstract: OBJECTIVE
  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].
  
  
  CONCLUSIONS
  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.
authors.affiliation: 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.
authors.forename: Benjamin
authors.initials: B
authors.lastname: Terrier
authors.affiliation: Department of Epidemiology, INSERM, U1153, Hôtel Dieu, AP-HP, Paris.
authors.forename: Agnès
authors.initials: A
authors.lastname: Dechartres
authors.affiliation: Department of Internal Medicine, Hôpital Edouard Herriot, Lyon.
authors.forename: Charlotte
authors.initials: C
authors.lastname: Girard
authors.affiliation: Department of Respiratory Diseases, Hôpital Pontchaillou, IRSET UMR 1085, Université de Rennes 1, Rennes, 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.
authors.forename: Stéphane
authors.initials: S
authors.lastname: Jouneau
authors.affiliation: Department of Internal Medicine, Hôpital Foch, Suresnes.
authors.forename: Jean-Emmanuel
authors.initials: JE
authors.lastname: Kahn
authors.affiliation: Department of Internal Medicine, Hôpital Avicenne, Bobigny.
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.affiliation: Department of Internal Medicine, Hôpital Haut-Lévêque, Bordeaux.
authors.forename: Estibaliz
authors.initials: E
authors.lastname: Lazaro
authors.affiliation: Department of Internal Medicine, Hôpital Saint-Antoine.
authors.forename: Jean
authors.initials: J
authors.lastname: Cabane
authors.affiliation: Department of Internal Medicine, Hôpital Bichat, Paris.
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
authors.affiliation: Department of Internal Medicine, Hôpital Conception, Marseille.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.affiliation: 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.
authors.forename: Pascal
authors.initials: P
authors.lastname: Cohen
authors.affiliation: Department of Dermatology, Centre Hospitalier, Pontoise.
authors.forename: Edouard
authors.initials: E
authors.lastname: Begon
authors.affiliation: Department of Internal Medicine, Hôpital La Timone, Marseille.
authors.forename: Pauline
authors.initials: P
authors.lastname: Belenotti
authors.affiliation: Department of Nephrology, Hôpital Rangueil, Toulouse.
authors.forename: Dominique
authors.initials: D
authors.lastname: Chauveau
authors.affiliation: Department of Internal Medicine, Hôpital Bretonneau, Tours.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.affiliation: Department of Internal Medicine, SELARL Loire Intermed, nouvelles cliniques nantaises.
authors.forename: Thierry
authors.initials: T
authors.lastname: Généreau
authors.affiliation: Department of Internal Medicine, Hôtel Dieu, Nantes.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Hamidou
authors.affiliation: Department of Rheumatology, Hôpital Bichat, Paris.
authors.forename: Gilles
authors.initials: G
authors.lastname: Hayem
authors.affiliation: Department of Internal Medicine, Hôpital Estaing, Clermont-Ferrand.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Le Guenno
authors.affiliation: 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.
authors.forename: Véronique
authors.initials: V
authors.lastname: Le Guern
authors.affiliation: Department of Internal Medicine, Hôpital Henri Mondor, Créteil.
authors.forename: Marc
authors.initials: M
authors.lastname: Michel
authors.affiliation: Department of Internal Medicine, Hôpital Purpan, Université de Toulouse.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Moulis
authors.affiliation: 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.
authors.forename: Xavier
authors.initials: X
authors.lastname: Puéchal
authors.affiliation: Department of Internal Medicine, Hôpital Lapeyronnie, Montpellier.
authors.forename: Sophie
authors.initials: S
authors.lastname: Rivière
authors.affiliation: Department of Internal Medicine and Clinical Immunology, Hôpital du Bocage, Dijon.
authors.forename: Maxime
authors.initials: M
authors.lastname: Samson
authors.affiliation: Department of Pneumonology and Thoracic Surgery, Hôpital Foch, Suresnes and.
authors.forename: François
authors.initials: F
authors.lastname: Gonin
authors.affiliation: 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.
authors.forename: Claire
authors.initials: C
authors.lastname: Le Jeunne
authors.affiliation: Department of Otolaryngology, Hôpital Cochin, AP-HP, Université Paris Descartes, Paris, France.
authors.forename: Pascal
authors.initials: P
authors.lastname: Corlieu
authors.affiliation: 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.
authors.forename: Luc
authors.initials: L
authors.lastname: Mouthon
authors.forename: Loic
authors.initials: L
authors.lastname: Guillevin
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001982:Bronchial Diseases / Q000209:etiology* / Q000628:therapy*; D003251:Constriction, Pathologic / Q000209:etiology / Q000628:therapy; D004106:Dilatation / Q000379:methods; D004724:Endoscopy / Q000379:methods*; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications*; D006801:Humans; D007267:Injections; D053685:Laser Therapy / Q000379:methods; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D015607:Stents; D013256:Steroids / Q000008:administration & dosage / Q000627:therapeutic use; D014135:Tracheal Stenosis / Q000209:etiology* / Q000628:therapy*; D017211:Treatment Failure; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D013256:Steroids
keywords: bronchial stenosis; dilatation; granulomatosis with polyangiitis; prosthesis; subglottic stenosis; tracheobronchial involvement