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Published online: 2024-01-24

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Subglottic stenosis of the larynx in granulomatosis with polyangiitis. A case report and review of the available literature

Ewelina Machura-Porębska1, Przemysław Kotyla1, Robert Pieczyrak1, Magdalena Kopeć-Mędrek1, Barbara Buc-Piorun1, Klaudia Palka1

Abstract

Granulomatosis with polyangiitis (GPA) is a rare systemic connective tissue disease marked by necrotising granulomatous inflammation and inflammation of small and medium-sized vessels. Due to its varied clinical presentation, GPA presents a major challenge for both clinicians and patients. Subglottic stenosis of the larynx (SGS) is a rare manifestation of GPA and is observed in 16–23% of patients. The aetiopathogenesis of this phenomenon is not fully elucidated. There was no correlation between the presence of airway narrowing (stenosis) and the activity and course of the disease. Subglottic stenosis can be a life-threatening condition with a need for urgent tracheotomy. The treatment of choice is immunosuppressive therapy with follow-up surgical treatment, usually using endoscopic approaches. A case of a 46-year-old female patient with SGS in GPA is presented. The patient reported severe dyspnoea, reduction of exercise tolerance and speech impairment. Intravenous pulses of corticosteroids were administered urgently and current immunosuppressive treatment was intensified, achieving a significant improvement in the patient's general condition. 

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