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Diseases

Genetic and Rare Diseases Information Center (GARD)

Relapsing polychondritis


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.

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) is characterized by recurrent inflammation of cartilage (the tough but flexible tissue that covers the ends of bones at a joint) and other tissues throughout the body. The features of the condition and the severity of symptoms vary significantly from person to person, but may include:[1][2][3]
  • Ear: The ears are the most commonly affected body part. Symptoms include a sudden onset of pain, swelling, and tenderness of the cartilage of one or both ears. 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.
  • Joint: The second most common finding is joint pain with or without arthritis.
  • Eye: Affected people may experience episcleritis, uveitis and/or scleritis. Scleritis may lead to a bluish or dark discoloration of the sclera (white of the eye) and may even be associated with vision loss in severe cases. Proptosis (bulging out of one or both eye balls) may also be a symptom of RP.
  • Nose: Nasal cartilage inflammation may lead to stuffiness, crusting, rhinorrhea, epistaxis (nose bleeds), compromised sense of smell and/or saddle nose deformity (a condition where the nose is weakened and thus "saddled" in the middle).
  • Airways: Inflammation may affect the larynx, trachea (windpipe), and bronchi (tubes that branch off the trachea and carry air to the lungs). Airway involvement may lead to a cough, wheezing, hoarseness and recurrent infections. It can become life-threatening if not properly diagnosed and managed.

Less commonly, RP may affect the heart, kidneys, nervous system, gastrointestinal tract, and/or vascular (veins) system. Nonspecific symptoms such as fever, weight loss, malaise, and fatigue may also be present.[3]

In approximately one third of affected people, RP is associated with other medical problems. Conditions reportedly associated with RP include hematological disease (including Hodgkin's lymphoma and myelodysplastic syndromes); gastrointestinal disorders (including Crohn's disease and ulcerative colitis); endocrine diseases (including diabetes mellitus type 1 and thyroid disorders) and others.[1]

Episodes of RP may last a few days or weeks and typically resolve with or without treatment. However, it is generally progressive, and many people have persistent symptoms in between flares.[1]
Last updated: 4/21/2015

How might relapsing polychondritis be treated?

The primary goals of treatment for people 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 to decrease the severity, frequency and duration of relapses. Higher doses are generally given during flares, while lower doses can typically be prescribed during periods of remission. Other medications reported to control symptoms include dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine and infliximab.[4][5]

People who develop severe heart or respiratory complications may require surgery.[6]

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: 4/21/2015

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.