RELAPSING POLYCHONDRITIS

By Gary M. White, MD


Courtesy Michael O. Murphy, MD
Relapsing polychondritis

Relapsing polychondritis (RP) is an inflammatory condition in which the immune system mounts and attack against the cartilage of the ear and other body parts.

Clinical

Pain and swelling of the ears with sparing of the lobes is characteristic. The nose may be involved and over time, significant ear and nose deformity may occur. Recurrences are common. After years, the ears may become floppy.

Death from RP may occur from systemic involvement, e.g. cardiac or pulmonary complications or infection. Pericarditis may be a component of RP, and thus cardiac surveillance with echocardiogram should be performed at all stages of disease. Potential serious complications such as atrial flutter have been associated with acute pericarditis in RP.

Diagnosis

The diagnosis is made clinically. Several criteria have been proposed. For example McAdams [Medicine 1976:55;193-215] proposed the following needing at least 3 for diagnosis:

Treatment

Because of the potentially fatal complications, a multidisciplinary approach is critical. Prednisone 30-60 mg/day is usually effective. Pulse methylprednisolone (1000mg/day) has been used. Methotrexate at a dose of 17.5 mg/week was effective at reducing the need for steroids. Other treatment options include dapsone alone, dapsone with prednisone, cyclosporine, and cyclophosphamide with prednisone. Several TNF agents have been used with success.

Pediatric cases may be treated with prednisone, dapsone and even methotrexate if needed [PD 2002;19;60].

Additional Pictures

Relapsing polychondritis

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