An idiopathic disorder characterized by recurrent inflammation of cartilaginous structures. Cardinal manifestations include ear and nose involvement with floppy ear and saddlenose deformities, inflammation and collapse of tracheal and bronchial cartilaginous rings, and asymmetric episodic nondeforming polyarthritis. Other features can include scleritis, conjunctivitis, iritis, keratitis, aortic regurgitation, glomerulonephritis, and other features of systemic vasculitis. Onset is frequently abrupt, with the appearance of 1-2 sites of cartilaginous inflammation. Diagnosis is made clinically and may be confirmed by biopsy of affected cartilage.
Treatment: Relapsing Polychondritis Glucocorticoids (prednisone 40-60 mg/d with subsequent taper) may suppress acute features and reduce the severity/frequency of recurrences. Cytotoxic agents should be reserved for unresponsive disease or for pts who require high glucocorticoid doses. When airway obstruction is severe, tracheostomy is required. |
Section 12. Allergy, Clinical Immunology, and Rheumatology