Section: 
Diseases of the lymphoepithelial pharyngeal ring and neck
Diseases of the lymphoepithelial pharyngeal ring and neckClinical variants of laryngeal and tracheal lesions in generalized and localized forms of granulomatosis with polyangiitis (Wegener’s disease). Part 2
I. A. Osipova (1), V. V. Vavin (2), P. I. Novikov (3)
(1) MEDEXPERT PLUS, LLC, Moscow, 121170, Russian Federation, (2) Endentika Medical Center, LLC, Moscow, 129323, Russian Federation, (3) Sechenov First Moscow State Medical University, Moscow, 11999(1), Russian Federation
UDK: УДК 616.22+616.31-007.271-002.7:616.13-002
DOI: https://doi.org/10.18692/1810-4800-2025-5-44-55
ABSTRACT
Abstract. The second part of the article presents data from our own observations of 41 patients with granulomatosis with polyangiitis (GPA) affecting the larynx and trachea as well as the results of medical and surgical treatment. It was shown that in 13 (65%) patients with the generalized form of GPA involving the larynx and trachea induction therapy using glucocorticosteroids and cyclophosphamide followed by maintenance immunosuppressive therapy failed to achieve disease remission, necessitating a switch to rituximab. Rituximab is likely to be more effective in preventing the progression of subglottic laryngeal stenosis (SGS) and tracheal stenosis (TS) in GPA, increases the success rate of surgical treatment, and reduces the likelihood of disease recurrence. The effectiveness of surgical treatment of SGS and TS in GPA depends on several factors. 1. The time elapsed from the onset of symptoms in the larynx and trachea to the start of immunosuppressive therapy. Early administration of immunosuppressive therapy improves the prognosis for subsequent restoration of the airway lumen. 2. Stages of disease activity: Surgical treatment at the highly active stage leads to recurrence of stenosis or deterioration of surgical results over time. Repeated surgical procedures performed at this stage lead to a worsening of the situation, fraught with severe, extended stenosis and patient disability. 3. Stage of drug treatment. During the induction therapy stage, disease activity remains, and salvage surgery may be required. Endoscopic intraluminal interventions may be the method of choice. Surgical treatment may be most effective during the remission stage, both endoscopic intraluminal interventions and staged surgical repair can be effectively performed. Tracheostomy, even as a life-saving surgical procedure, leads to a worse prognosis for airway restoration.
Publication date:
20.10.2025
Keywords:
granulomatosis with polyangiitis, generalized form, localized form, subglottic laryngitis, subglottic laryngeal stenosis, supraglottic laryngeal stenosis, tracheal stenosis For citation:
Osipova I. A., Vavin V. V., Novikov P. I. Clinical variants of laryngeal and tracheal lesions in generalized and localized forms of granulomatosis with polyangiitis (Wegener’s disease). Part 2. Российская оториноларингология. 2025;24(5):44–55. https://doi.org/10.18692/1810-4800-2025-5-44-55