TABLE 2762 Respiratory Manifestations of Rheumatic Diseases

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Rheumatoid arthritis (RA) may cause cricoarytenoid dysfunction. RA patients with cricoarytenoid arthritis may complain of pain with speaking or swallowing, hoarseness, or stridor. If the joints become fixed in a closed position, airway compromise mandates emergency tracheostomy. Presentation with the above signs is an urgent indication for fiberoptic laryngoscopy.

The respiratory muscles may be impaired by inflammatory muscle disease. In dermatomyositis and polymyositis, respiratory insufficiency is uncommon at presentation but complicates poorly controlled or chronic disease. Patients should be observed for nasal flaring, as they may be too weak to generate intercostal retractions. Chest x-rays are normal or show "high-riding" diaphragms unless pulmonary involvement or an aspiration pneumonitis coexists. Admission for disease control and stabilization is indicated in any patient with inflammatory muscle disease and respiratory impairment. Patients with active or advanced disease may require intubation and ventilation for respiratory support. The clinical course can be followed at bedside with measurements of peak inspiratory and expiratory forces. Respiratory failure is predictable with peak inspiratory effort of less than 30 percent of predicted or vital capacity of less than 50 percent of predicted.

Pleurisy is common in RA and systemic lupus erythematosus (SLE). It may be asymptomatic in RA, but roughly half of lupus patients have signs and symptoms of pleurisy during the course of their disease. All effusions in rheumatic disease patients should be aspirated for diagnosis. Rheumatoid pulmonary effusions characteristically have very low glucose levels, but so do effusions associated with indolent infections such as tuberculosis. Rheumatic, infectious, and malignant effusions are often exudative and either polymorphonuclear or mononuclear cells may predominate. In the patient without lupus nephritis, nonsteroidal anti-inflammatory drugs (NSAIDs) (e.g., indomethacin, 25 to 50 mg tid) usually suffice. Prednisone in moderate doses 10 mg po tid is reserved for patients failing this regimen.

Pulmonary hemorrhage can complicate Goodpasture's disease, SLE, hypersensitivity vasculitis, and Wegener's granulomatosis due to vasculopathic or vasculitic lung involvement. Ankylosing spondylitis (upper lobes), scleroderma, and, rarely, RA and other rheumatic diseases can lead to pulmonary fibrosis. Patients may present with abrupt decompensation with a history of a well-tolerated slow decline. In myositis, however, interstitial pneumonitis may become apparent with quiescent muscle disease, progress rapidly, and result in fulminant respiratory failure; these patients should be hospitalized to rule out infection and be treated with aggressive immunosuppression.

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