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Dx: Relapsing Polychondritis

This patient had relapsing polychondritis, a rare autoimmune disease that affects the cartilaginous structures of the body. Structures at risk include the nose, ears, joints and the tracheobronchial tree. Also at risk are the eyes, heart and blood vessels.

altAlmost all patients have auricular involvement at some point in their course. Eye symptoms, including scleritis, episcleritis, conjunctivitis, keratitis or uveitis, occur in half of the patients. Relapsing polychondritis is a clinical diagnosis. The differential varies based on which structures are affected. Wegener’s granulomatosis may present with nasal destruction. Auricular swelling may also be caused by trauma or infection.

altTreatment for relapsing polychondritis varies based on the severity and location of inflammation. Chondritis localized to the ears or nose may be treated with nonsteroidal anti-inflammatory drugs. More serious organ involvement is treated with steroids, other immunosuppressive drugs and recently with emerging biological therapies. Maintenance therapy has been attempted with cochicine, indomethacin, or diaminodiphenylsulfone (dapsone) with some reports of success. Surgical intervention for airway and cardiac involvement may be necessary.

Patients with relapsing polychondritis have a reduced life expectancy. Symptoms fluctuate but typically progress. Destruction of respiratory and cardiac structures lead to the significant morbidity and mortality associated with the disease.

 

References:
Ernst A. Relapsing polychondritis and airway
     involvement. Chest. 2009 Apr; 135(4):1024-30.
Gergely P. Relapsing polychondritis. Best Pract Res Clin
     Rheumatol. 2004 Oct. 18(5):723–38.
Trentham DE. Relapsing polychondritis. Ann Intern
     Med. 1998 Jul; 129(2):114-22.

 

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