Clinical outcomes of subglottic stenosis in granulomatosis with polyangiitis: results of an international multicentre observational study
Year: 2026
Type: article
Study design: Observational Study
Abstract: OBJECTIVES: Subglottic stenosis (SGS) is a challenging manifestation of granulomatosis with polyangiitis (GPA), often relapsing and poorly responsive to immunosuppressive treatment. Current guidelines lack specific recommendations for SGS management and evidence is limited. This study aimed to identify features of SGS associated with a more aggressive course and to assess the efficacy of available treatments in preventing relapses. METHODS: We conducted a multicentre, retrospective cohort study including GPA patients with SGS. Patients were stratified into higher relapsers (≥2 flares) and lower relapsers (≤1 flare). Clinical and treatment features were compared across groups. Univariate and multivariate analyses were conducted to identify independent predictors of relapse and multiple flares. Kaplan-Meier curves assessed time-to-relapse across regimens. RESULTS: Eighty-nine patients were included (30% male). Forty-eight patients (54%) were higher relapsers, with a median time-to-relapse of 36 months. Systemic immunosuppressive therapy was associated with fewer relapses (82% vs 18%, P = 0.04) compared with local treatments alone. Glucocorticoids in induction regimens reduced relapse risk (86% vs 12%, P = 0.03). Cyclophosphamide (CYC) was associated with the longest relapse-free survival and reduced 5-year relapse risk (HR 0.33, P = 0.049). By contrast, glucocorticoid monotherapy in either induction or maintenance phase was associated with higher relapse rate (P = 0.006). No specific maintenance regimen was significantly protective, though rituximab and DMARDs showed a trend toward improved outcomes. CONCLUSION: Systemic immunosuppressive therapy, particularly CYC-based induction, was associated with fewer SGS relapses and prolonged relapse-free survival, while glucocorticoid monotherapy was less effective. Prospective studies are needed to optimize induction and maintenance strategies in GPA-related SGS.
Source: Lara D. Veeken
Authors: Gabriele Domenico Gallina, Marco Lanzillotta, Sara Maggioni, Adriana Cariddi, Giulia Danè, Ayşe Nur Bayındır Akbaş, Askin Ates, Alvise Berti, Enrico Brunetta, Ayse Cefle, Emanuele Chiara, Edoardo Conticini, Vincent Cottin, Paolo Delvino, Filippo Fagni, Gözde Sevgi Kart Bayram, Ömer Karadağ, Gabriella Moroni, Sergey Moiseev, Sara Monti, Tahir Saygın Öğüt, Pavel Novikov, Roberto Padoan, Luca Iorio, Federica Davanzo, Michelangelo Tesi, Luca Quartuccio, Ayten Yazici, Marco Matucci-Cerinic, Lorenzo Dagna, Luca Moroni, the STOP-GPA Study Group, Veli Cobankara, Veli Yazisiz, Emine Uslu, Servet Akar, Hasan Kocaayan, Hamit Kucuk, Rıza Can Kardaş, Timucin Kasifoglu, Reşit Yıldırım, Muhammed Emin Kutlu, Fatos Onen, Tuba Demirci, Mehmet Engin Tezcan, Duygu Şahin, Ahmet Omma, Pınar Akyüz Dağlı, Alessandra Milanesi, Elena Treppo, Giacomo Emmi, Stefano Viscardi, Umberto Tanzini, Apostolos Karligkiotis, Pierpaolo Lattanzi, Salvatore Carrabino, Roberto Bortolotti, Francesco Peyronel, Camille Taille Hôpital Bichat, Gregory Pugnet, Biancamaria Venerandi
Institutions: IRCCS Ospedale San Raffaele, Vita-Salute San Raffaele University, Pamukkale University, Ankara University, University of Trento, Ospedale Santa Chiara, Humanitas University, IRCCS Humanitas Research Hospital, Kocaeli Üniversitesi, University of Trieste, Ospedale di Cattinara, University of Siena, Lyon 1 Université, Hôpital Louis Pradel, Azienda Ospedaliera San Gerardo, University of Milano-Bicocca, Universitätsklinikum Erlangen, Hacettepe University, Sechenov University, University of Milan, IRCCS Istituto Auxologico Italiano, Akdeniz University, University of Padua, Meyer Children's Hospital, University of Udine, Azienda sanitaria universitaria Friuli Centrale
Language: English
Subfield: Pulmonary and Respiratory Medicine
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W7131246973