Prognosis

The prognosis of RP varies, depending upon the pattern and severity of chondral inflammation. Most patients (84%) will have intermittent flares lasting days to weeks (18). Many patients have chronic indolent symptoms between flares, and most will develop some degree of disability. A minority of patients will have a benign course free of visceral involvement or a fulminant course resulting in death. The most common complications are loss of hearing and olfaction, reduced vision (8%), phonation difficulties, and neurologic dysfunction. Potentially life-threatening complications include airway compromise from tracheal narrowing or collapse (14%), aortic or mitral valvular incompetence or rupture (8%), heart block, aortic involvement, and arterial or venous thrombosis. Earlier studies showed an overall survival rate of 74% at 5 years and 55% at 10 years (18). More recent studies show an improved survival rate of 94% at eight years, perhaps reflecting better medical and surgical therapy (20).

Pneumonia is the most common cause of death, followed by respiratory failure from airway collapse and complications of valvular heart disease and vasculitis. Poor prognostic factors predicting decreased survival include younger age (less than 50 years of age at onset), saddle nose deformity, arthritis, tracheolaryngeal involvement, renal disease, and cardiovascular inflammation including valve involvement, aortic disease, and vasculitis (18). Of note is that patients frequently have associated diseases including systemic vasculitis, various connective tissue diseases, neoplastic disease (particularly myelodys-plastic syndrome), and others that may affect the prognosis.

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