Meaningful clues and physical signs in chest disease.
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Biomedical subjects
Publications and source records attributed to R A DeRemee.
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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.
Review of 112 patients with Wegener's granulomatosis showed that 21 (19%) had ear involvement. Conductive deafness, which was present in all 21 patients, was due to serous middle ear fluid, suppurative otitis media with thickening of the tympanic membrane, perforation of the tympanic membrane, or granulation tissue in the middle ear space. Nine patients also had sensorineural hearing loss. Sensorineural hearing loss was improved in five of the nine patients after control of the disease with prednisone and cyclophosphamide.