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Genetic and Rare Diseases Information Center (GARD)

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Relapsing polychondritis


Other Names for this Disease

  • Chronic atrophic polychondritis
  • Recurrent polychondritis
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Your Question

My wife was diagnosed with relapsing polychondritis over 6 years ago. Her ear flares up about 2-3 times a year. In the beginning it went away on its own; now she takes prednisone but only during the flare. Our doctor says the next step would be methotrexate but before that she'd be on daily prednisone. The disease doesn't seem to be progressing and CT and PET scans indicate no other damage. Is there something more we should be doing such as diet supplements or other medications? Are there other things we should be watching for?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What are the signs and symptoms of relapsing polychondritis?

Relapsing polychondritis (RP) has been associated with a wide variety of conditions, signs and symptoms. Individuals with RP generally have a sudden onset of symptoms, typically involving the external ear and experiencing pain, swelling, redness and tenderness. Episodes may last a few days or weeks and typically resolve with or without treatment. The pinna usually loses firmness and becomes floppy; hearing impairment may also occur. Inflammation of the inner ear may also cause nausea, vomiting, dizziness, and/or ataxia. The second most common finding is joint pain with or without arthritis. About a third of affected individuals have inflammation of the blood vessels (vasculitis) and autoimmune rheumatic diseases such as rheumatoid arthritis and lupus. Ocular (eye) findings occur in about 60% and may include inflammation or irritation of various parts of the eye(s) and/or other findings. Respiratory symptoms and non-specific skin disorders (present in about 50% of affected individuals) are also common. Other findings that have been reported, but occur less commonly, include neurological abnormalities, cardiovascular (heart) manifestations and renal disease; renal disease typically indicates a worse prognosis. Other conditions reportedly associated with RP include hematological disease (including Hodgkin's lymphoma, myelodysplastic syndromes and others); gastrointestinal disorders (including Crohn's disease, ulcerative colitis and others); endocrine diseases (including diabetes mellitus type 1 and thyroid disorders) and others. RP is generally progressive, and many individuals have persistent symptoms in between flares. Most affected individuals develop some degree of disability over time.[1]
Last updated: 12/19/2011

How might relapsing polychondritis be treated?

The primary goals of treatment for individuals with relapsing polychondritis (RP) are to relieve present symptoms and to preserve the structure of the affected cartilage. The main treatment for RP is corticosteroid therapy with prednisone, with higher doses during flares and lower doses during periods of remission to decrease the severity, frequency and duration of relapses.[2] Other medications reported to control symptoms include dapsone, azathioprine, methotrexate, cyclophosphamide, and cyclosporin A. Methotrexate in conjunction with steroids has reportedly been found to significantly decrease the need for corticosteroids while controlling symptoms. Other medications that have shown benefit include anakinra, leflunomide, rituximab and anti–tumor necrosis factor-alpha inhibitors (typically used to treat autoimmune diseases).[2] Individuals who develop severe heart or respiratory complications may require surgery.[1] No specific dietary recommendations have been noted.[2]

More detailed information about the management of RP is available on Medscape Reference's Web site and can be viewed by clicking here.
Last updated: 6/10/2013

References
Other Names for this Disease
  • Chronic atrophic polychondritis
  • Recurrent polychondritis
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.