Case Reports
30 July 2025
Early Access
Successful treatment of refractory tracheal stenosis complicating anti-neutrophil cytoplasm antibody-associated vasculitis with sirolimus
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All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. Any product that may be evaluated in this article or claim that may be made by its manufacturer is not guaranteed or endorsed by the publisher.
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Laryngotracheal granulomatous inflammation is a clinical entity that may complicate either localized or systemic disorders. It can result in life-threatening airway compromise, requiring urgent medical or surgical intervention. We report the case of a patient presenting with recurrent tracheal stenosis secondary to anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis, refractory to conventional treatments. Despite multiple endoscopic dilatations and surgical resection of tracheal rings, disease control remained suboptimal. Introduction of sirolimus led to a sustained improvement of the stenotic lesion, as demonstrated by clinical and instrumental assessment. Sirolimus may be considered a valuable therapeutic option for severe subglottic inflammatory manifestations in ANCA-associated vasculitis.
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Aravena C, Almeida FA, Mukhopadhyay S, Ghosh S, Lorenz RR, Murthy SC, et al. Idiopathic subglottic stenosis: a review. J Thorac Dis 2020; 12: 1100-11. DOI: https://doi.org/10.21037/jtd.2019.11.43
Mokhtari TE, Miller LE, Jayawardena ADL, Sadow PM, Hardy SC, Rothman D, et al. Eosinophilic granulomatosis with polyangiitis: an unusual case of pediatric subglottic stenosis. Laryngoscope 2021; 131: 656-9. DOI: https://doi.org/10.1002/lary.28848
Bach Á, Ambrus A, Iványi B, Tóbiás Z, Alim Marvasti GH, Rovó L. IgG4-related laryngeal disease as a possible cause of idiopathic subglottic stenosis: a case report. Iran J Otorhinolaryngol 2021; 33: 119-25.
Lee PY, Adil EA, Irace AL, Neff L, Son MBF, Lee EY, et al. The presentation and management of granulomatosis with polyangiitis (Wegener’s Granulomatosis) in the pediatric airway. Laryngoscope 2017; 127: 233-40. DOI: https://doi.org/10.1002/lary.26013
Colevas SM, Gietman BT, Cook SM, Kille TL. Granulomatous inflammation causing severe supraglottic edema and airway obstruction in a pediatric patient. Ear Nose Throat J 2022; 101: NP375-8. DOI: https://doi.org/10.1177/0145561320973772
Solans-Laqué R, Bosch-Gil J, Canela M, Lorente J, Pallisa E, Vilardell-Tarrés M. Clinical features and therapeutic management of subglottic stenosis in patients with Wegener’s granulomatosis. Lupus 2008; 17: 832-6. DOI: https://doi.org/10.1177/0961203308089693
Fijolek J, Wiatr E, Gawryluk D, Martusewicz-Boros MM, Orlowski TM, Dziedzic D, et al. Intratracheal dilation-injection technique in the treatment of granulomatosis with polyangiitis patients with subglottic stenosis. J Rheumatol 2016; 43: 2042-8. DOI: https://doi.org/10.3899/jrheum.151355
Lebovics RS, Hoffman GS, Leavitt RY, Kerr GS, Travis WD, Kammerer W, et al. The management of subglottic stenosis in patients with Wegener’s granulomatosis. Laryngoscope 1992; 102: 1341-5. DOI: https://doi.org/10.1288/00005537-199212000-00005
Geetha D, Jefferson JA. ANCA-associated vasculitis: core curriculum 2020. Am J Kidney Dis 2020; 75: 124-37. DOI: https://doi.org/10.1053/j.ajkd.2019.04.031
Monti S, Felicetti M, Delvino P, Padoan R, Berti A, Paolazzi G et al. Anti-neutrophil cytoplasmic antibody specificity determines a different clinical subset in granulomatosis with polyangiitis. Clin Exp Rheumatol 2021; 39: 107-13. DOI: https://doi.org/10.55563/clinexprheumatol/50919f
Schirmer JH, Wright MN, Herrmann K, Laudien M, Nölle B, Reinhold-Keller E et al. Myeloperoxidase-antineutrophil cytoplasmic antibody (ANCA)-positive granulomatosis with polyangiitis (Wegener's) is a clinically distinct subset of ANCA-associated Vasculitis: a retrospective analysis of 315 patients from a German vasculitis referral center. Arthritis Rheumatol. 2016; 68: 2953-63. DOI: https://doi.org/10.1002/art.39786
Poo SX, Pepper R, Onwordi L, Ghufoor K, Sandhu G, Salama A. Sirolimus use in patients with subglottic stenosis in the context of granulomatosis with polyangiitis (GPA), suspected GPA, and immunoglobulin G4 -related disease. Scand J Rheumatol 2021; 50: 52-7. DOI: https://doi.org/10.1080/03009742.2020.1777324
Samtani P, Li M, Soma M, Teng A, Jacobson I, Banks C, et al. Idiopathic laryngeal granulomatosis in a teenage girl responsive to sirolimus. Otolaryngology Case Reports 2024; 31: 100599. DOI: https://doi.org/10.1016/j.xocr.2024.100599
Namba DR, Ma G, Samad I, Ding D, Pandian V, Powell JD, et al. Rapamycin inhibits human laryngotracheal stenosis–derived fibroblast proliferation, metabolism, and function in vitro. Otolaryngol Head Neck Surg 2015; 152: 881-8. DOI: https://doi.org/10.1177/0194599815573708
Motz KM, Lina IA, Samad I, Murphy MK, Duvvuri M, Davis RJ, et al. Sirolimus-eluting airway stent reduces profibrotic Th17 cells and inhibits laryngotracheal stenosis. JCI Insight 2023; 8: e158456. DOI: https://doi.org/10.1172/jci.insight.158456
Moroni L, Giudice L, Lanzillotta M, Cariddi A, Ramirez GA, Bozzolo EP et al. Role of systemic immunosuppression on subglottic stenosis in granulomatosis with polyangiitis: analysis of a single-centre cohort. Eur J Intern Med 2023; 114: 108-12. DOI: https://doi.org/10.1016/j.ejim.2023.05.006
Monach PA. L25. Medical treatment of subglottic stenosis in granulomatosis with polyangiitis (Wegener's). Presse Med 2013; 42: 575-6. DOI: https://doi.org/10.1016/j.lpm.2013.01.025
Aden AA, Awadallah AS, Xie KZ, Wallerius KP, O’Byrne TJ, Bowen AJ, et al. Medical maintenance therapy following laser excision in patients with granulomatosis with polyangiitis (GPA)-associated subglottic stenosis. Otolaryngol Head Neck Surg 2024; 171: 180-7. DOI: https://doi.org/10.1002/ohn.694
Almuhanna A, Almahboob A, Alhussien A, Aljurayyed R, Alammar A. Current therapeutic approaches to subglottic stenosis in patients with GPA: a systematic review. Ear Nose Throat J 2024; 103: 117-25. DOI: https://doi.org/10.1177/01455613211036246
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Successful treatment of refractory tracheal stenosis complicating anti-neutrophil cytoplasm antibody-associated vasculitis with sirolimus. (2025). Reumatismo. https://doi.org/10.4081/reumatismo.2025.1851
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