By Gary M. White, MD
Courtesy Michael O. Murphy, MD
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.
- Arthralgias, episcleritis, cardiac valvular involvement and potentially life-threatening pulmonary disease with tracheal stenosis may occur.
- Antibodies to types II, IX and XI cartilage have been documented. Urine mucopolysacharides maybe elevated during flares.
- An increased incidence of myelodysplastic syndrome has been reported.
- Very rarely, patients may have features of Behcet's Syndrome as well (e.g. oral and genital ulcers), called MAGIC Syndrome.
- At least 35 cases of RP-associated with meningoencephalitis have been reported [Cutis 2017;99;43]. Patients may present with fever, headache, altered mental status, dysarthria, agraphia and hemiparesis.
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.
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:
- Recurrent chondritis of both auricles
- Nonerosive inflammatory polyarthritis
- Chondritis of nasal cartilages
- Inflammation of ocular structures
- Chondritis of the respiratory tract
- Cochlear and/or vestibular damage
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].
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