Relapsing polychondritis.

Abstract

Relapsing polychondritis is a rare inflammatory disease of unknown aetiology characterized by recurrent inflammation and destruction of cartilaginous structures and connective tissue. Current data provide increasing support for an autoimmune basis, but its cause remains unknown. Individuals of any race, gender, or age may be affected, but it is most commonly seen between the ages of 40 and 60 years. Although relapsing polychondritis occurs predominantly as a separately defined clinical complex, a significant number of patients may suffer from another underlying rheumatic and/or haematological disorder; vasculitic syndromes are the most commonly observed disorders associated with relapsing polychondritis. Common clinical features are auricular, nasal and respiratory tract chondritis with involvement of organs of special sense, such as the eyes and audiovestibular apparatus. Polyarthritis and vasculitic involvement are also common. Corticosteroids are still the agents of choice although several other anti-inflammatory drugs can be used in order to allow tapering of the steroid dose or to achieve a lower maintenance dose for refractory cases.