Section: 
Diseases of the lymphoepithelial pharyngeal ring and neck
Diseases of the lymphoepithelial pharyngeal ring and neckLaryngeal pathology in granulomatosis with polyangiitis
Yu. E. Stepanova (1), E. E. Koren’ (2)
(1), (2) Saint Petersburg Research Institute of Ear, Throat, Nose and Speech, Saint Petersburg, 190013, Russian Federation
UDK: УДК 616.225-002.7-089.87:615.849.15
DOI: https://doi.org/10.18692/1810-4800-2025-6-14-21
ABSTRACT
Abstract. Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease of the ANCA-associated systemic vasculitis (ANCA-SV) group characterized by granulomas in the respiratory tract and systemic necrotizing vasculitis. The clinical manifestations of GPA in the larynx are insufficiently studied, and there is no description of the stages of cicatrical stenosis and their endophotography in the literature. In the clinical case presented in the article, various etiological factors for the development of granulomatosis with polyangiitis were identified in female patient A. born in 1991: genetic predisposition, allergy to dust, pollinosis, carriage of Staphylococcus aureus from childhood. 17 years has passed from the onset of the first signs of granulomatous polyangiitis to the formation of chronic cicatricial laryngeal stenosis, and less than 6 years has passed since the first complaints of shortness of breath appeared. During this period, the patient’s clinical manifestations of Wegener’s granulomatosis in the larynx have changed, which have passed through the following stages: 1) diffuse laryngeal stenosis: granulomatous infiltrates in the interglottic region and circular narrowing of the mucous membrane in the subglottic region of the larynx; 2) regression of infiltrates in the interglottic region and circular narrowing of the mucous membrane in the subglottic region of the larynx; 3) formation of a scar of the laryngeal-tracheal junction; 4) circular scar of the subglottic area of the larynx, single granulomas in the lumen of the trachea; 5) chronic cicatrical stenosis of the subglottic region of the larynx with two circular openings, obstruction of the tracheal lumen by granulomas. Throughout the years of follow-up, the patient was constantly receiving both traditional medications (glucocorticoids and cyclophosphamide) and modern genetically engineered biological anti-B-cell therapy drugs. This made it possible to increase the periods of remission, delay the formation of chronic laryngeal stenosis, and thereby improve the patient’s quality of life. This clinical case justifies the need for interdisciplinary collaboration between ENT specialists and rheumatologists as well as the timely use of endoscopic techniques to diagnose and monitoring treatment effectiveness.
Publication date:
23.12.2025
Keywords:
vasculitis, Wegener’s granulomatosis, granulomatosis with polyangiitis, dysphonia, hoarseness, cicatricial laryngeal stenosis For citation:
Stepanova Yu. E., Koren’ E. E. Laryngeal pathology in granulomatosis with polyangiitis. Russian Otorhinolaryngology. 2025;24(6):14-21. (In Russ.) https://doi.org/10.18692/1810-4800-2025-6-14-21