Relapsing polychondritis (RP) is a rare disease that occurs worldwide in all age groups and affects men and women equally.
An associated rheumatologic or hematologic disorder is seen in over 30% of patients with RP. Inflammation of cartilaginous structures with lymphocytic infiltration and association with anticollagen antibodies are consistent with an autoimmune pathogenesis.
The characteristic clinical findings are acute painful swelling and redness of the external ear, nasal cartilage, and laryngotracheal cartilage. Nonerosive arthritis, ocular inflammation, vestibular symptoms, and involvement of the heart and kidney also occur.
Diagnosis is made on clinical grounds, though cartilage biopsy may be useful.
Treatment with nonsteriodal anti-inflammatory drugs (NSAIDs) or corticosteroids may control inflammation, but immunosuppressive and biologic treatments may be required for severe disease.
■ Laryngotracheal involvement is a serious complication that may require tracheostomy.
KeywordsAortic Valve Disease Relapse Polychondritis Retinal Vasculitis Large Vessel Disease Nasal Cartilage
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