Abstract

Background: Subglottic stenosis is a well-known potentially life-threatening presentation of granulomatosis with polyangiitis (GPA). Circumferential scarring and critical narrowing of the airway at the level of cricoid cartilage and/or upper tracheal rings appears in 10–16% of patients with GPA. Symptoms range from cough and shortness of breath to life-threatening dyspnoea with stridor. Majority of patients need tracheostomy at the moment of diagnosis. Surgical treatment consists of endoscopic surgery, complex resection of the stenotic segment followed by reconstruction and stenting. Surgical therapy usually has to be repeated due to restenosis of the airway despite ongoing medical treatment. Endoscopic balloon dilatation is a procedure of choice in our institution in a long term management of those patients with good postoperative results. We present four patients with a long history of GPA complicated by airway obstruction and their management.

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