Wegener's granulomatosis of the subglottis and the upper portion of the trachea.
A relatively little-known complication of Wegener's granulomatosis is involvement of the subglottis and upper part of the trachea, which is illustrated in this report on 17 patients. Each of the patients had biopsy-proved Wegener's granulomatosis of the nose and paranasal sinuses. Some had concomitant involvement of the eye, kidney, lung and skin. Nine of the 17 patients required tracheotomy; 5 still require a tracheotomy tube, and 4 have had decannulation (1 had successful reconstructive surgery of the trachea). Medical treatment consisted of cyclophosphamide, with or without corticosteroids. All 17 are currently alive, the average follow-up having been 81 months. The otolaryngologist plays a key role in the detection and management of patients with Wegener's granulomatosis, particularly when the onset is a sudden progressive upper tracheal obstruction with airway symptoms that may overshadow the sometimes more subtle nasal manifestations.
D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D000368:Aged; D002648:Child; D003520:Cyclophosphamide / Q000627:therapeutic use; D003937:Diagnosis, Differential; D005260:Female; D005931:Glottis; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D006801:Humans; D007818:Laryngeal Diseases / Q000188:drug therapy / Q000601:surgery / Q000628:therapy; D008297:Male; D008875:Middle Aged; D014135:Tracheal Stenosis / Q000188:drug therapy / Q000601:surgery / Q000628:therapy*; D014140:Tracheotomy
D000305:Adrenal Cortex Hormones; D003520:Cyclophosphamide