Manifestations of Wegener’s granulomatosis involving the airways:
(1) subglottic stenosis (at the level of the cricoid cartilage)
(2) tracheal stenosis (usually at the level of the upper tracheal rings)
(3) bronchial stenosis
(4) laryngitis, tracheitis and/or bronchitis
Other manifestations may be present at onset, but sometimes the airway involvement may be the first or only sign of the vasculitis.
Clinical findings:
(1) cough
(2) hemoptysis
(3) stridor
(4) sibilant rales
(5) dyspnea
Detection of proteinase 3-ANCA can be helpful in establishing the diagnosis.
The airway involvement often follows a chronic, relapsing course. In these patients repeated treatments are necessary.
Therapy may take the form of:
(1) diltatation tracheoscopy and/or bronchoscopy
(2) endoscopic laser surgery
(3) other surgical interventions
