5.6 Laryngeal Manifestations of Systemic Diseases
Key Features
Systemic diseases may generate vocal and airway consequences.
Management of the underlying systemic illness may or may not enable reversal of the laryngeal issues. Systemic diseases may manifest as laryngeal symptoms and findings. A careful clinical history and appropriate testing should yield a diagnosis. Frequently, consultation with colleagues from other services more familiar with the overall systemic illness may be needed.
Rheumatoid Arthritis
Rheumatoid arthritis is a chronic, inflammatory condition affecting synovial joints with progressive arthritis and deformity. All synovial joints are vulnerable, with hands and feet the most commonly affected. Women are three times more likely to develop the disease, usually between the third and seventh decade. A juvenile form of the disease also exists. The cricoarytenoid joint may be involved in this process in 25 to 50% of those with long-standing disease. Symptoms may include hoarseness, globus, and airway compromise. Examination may show inflammatory changes of the arytenoid region, diffuse laryngeal myositis, and rheumatoid nodules within the vocal folds or unilateral or bilateral vocal fold motion impairment. Treatment may include nonsteroidal anti-inflammatory drugs (NSAIDs) or corticosteroids. Local injections of steroids into the cricoarytenoid joint region have shown success in improving joint mobility.
Relapsing Polychondritis
Relapsing polychondritis is a chronic and recurrent autoimmune inflammatory condition affecting all cartilage subtypes. The laryngotracheal complex may be involved in 40 to 50% of patients. Disease may affect the airways focally or diffusely. Symptoms may include hoarseness, dyspnea, stridor, laryngotracheal tenderness, or dysphagia. Airway symptoms may be secondary to acute inflammatory and swelling, laryngotracheal collapse secondary to replacement of cartilage by fibrosis, or subglottic stenosis. Treatment is as necessary for any airway obstruction, but in general, therapy consists of corticosteroids and other immunosuppressive agents.
Wegener′s Granulomatosis
Wegener′s granulomatosis is an autoimmune disorder characterized by necrotizing granulomas and vasculitis. The upper and lower respiratory tract and renal systems are primarily affected, although vasculitis may occur anywhere. The larynx is involved primarily in the subglottic and upper tracheal region, and pathology is seen in the early phase of ~ 10 to 15% of patients. Biopsy may demonstrate the characteristic granulomas. Antineutrophilic cytoplasmic antibodies are elevated in 90% of cases. Airway management of the subglottic stenosis may be necessary and involve dilations, laser resection, or tracheotomy. Formal laryngotracheoplasty or tracheal resection should be reserved for chronic, nonactive disease.