Relapsing Polychondritis

Abstract

Relapsing polychondritis is a recurrent episodic autoimmune inflammation against collagen types II, IX, and XI, involving cartilages and proteoglycan-rich structures, such as the eye, heart, blood vessels, and inner ear. Ocular disease (50–67%): 30% can be the initial presentation of the disease. Most commonly it involves the episclera and sclera, but may affect any ocular structure. Auricular chondritis (83–95%): unilateral or bilateral auricular pain and swelling, associated with hearing loss, tinnitus, and vertigo from inner ear involvement. Nonerosive arthritis (52–85%): asymmetric and often migratory polyarthritis lasting weeks to months. Nasal chondritis (33–72%): sudden painful onset with mild epistaxis. Laryngotracheal disease (30–67%): varies from asymptomatic to life-threatening complications; hoarseness, aphonia, wheezing, inspiratory stridor, nonproductive coughing, dyspnea. Skin/mucosal disease (0–83%): nonspecific and range from aphthous ulcers, nodules on the limbs, to purpura and pustules. Cardiovascular disease (6–23%): chest pain, silent MI, arrhythmias, heart block, and syncope from aortitis. Neurological disease (0–10%): due to vasculitis of the peripheral nervous system or CNS; peripheral neuropathy, cranial nerve palsy, hemiplegia, and seizures.