Mucosal inflammation/edema Mass lesions of the vocal fold Mechanical limitation of vocal fold motion Laryngeal paralysis Neuromotor dysfunction Psychogenic factors with epistaxis and/or chronic sinusitis. The larynx is involved in 6% to 25% of cases, usually with exophytic granulation tissue in the subglottis and sometimes the vocal folds. The inflammation can progress to subglottic stenosis.
There is a male predominance and onset is most common in the fifth and sixth decades. The classic criteria for diagnosis of Wegener's include necrotizing granuloma of the upper or lower respiratory tract and focal, necrotizing glomerulonephritis with fibrinoid necrosis and thrombosis of capillary loops. More recently, the diagnosis is established by disease in at least two organ systems, with positive biopsy in at least one. Tissue biopsy is the most reliable means of diagnosis but may be obscured by necrosis. anti-nuclear antibody (ANA) may be positive, but anti-nuclear cytoplasmic antibody (C-ANCA) is a more sensitive serologic test. Not infrequently, Wegener' s is suspected as the cause of isolated idiopathic subglottic stenosis, but biopsies and serologic testing are negative.
Primary treatment of Wegener's granulomatosis is pharmacologic. Steroids are usually effective. Second-line therapy includes cytotoxic drugs. Medical therapy may keep the disease in check, but often the disease progresses. In systemic disease, death results from pulmonary and/or renal failure. Laryngeal stenosis may require endoscopic excision to relieve airway obstruction but may be complicated by scarring, with further voice impairment and recurrent obstruction (Fig. 1). Tracheotomy is an alternate way of relieving obstruction. Surgical management of stenosis and scarring may be attempted when there is no active disease, but it may be complicated by reactivation (1).
Wegener's granulomatosis is discussed in detail in Chapter 8.
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