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Diseases

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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Overview



What is relapsing polychondritis?

What are the signs and symptoms of relapsing polychondritis?

How might relapsing polychondritis be treated?


What is relapsing polychondritis?

Relapsing polychondritis is a condition that affects connective tissue, particularly cartilage. Cartilage is a tough but flexible tissue that covers the ends of your bones at a joint, and gives shape and support to other parts of your body. Early symptoms of relapsing polychondritis may include irritation, redness, and swelling (inflammation) of the ears, nose, and/or eye. A person may experience arthritis involving multiple joints as well.  Relapsing polychondritis may affect the inner ear, heart, and skin and can cause sensorineural deafness, vertigo, aortic valve regurgitation, mitral valve regurgitation, and aneurysms.[1]

Last updated: 1/6/2010

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.[2]
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.[3] 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).[3] Individuals who develop severe heart or respiratory complications may require surgery.[2] No specific dietary recommendations have been noted.[3]

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
  1. KR Jones. Infections and manifestations of systemic disease of the larynx. In: Cummings et al.,. Otolaryngology: Head & Neck Surgery, 4th ed. Philadelphia, PA: Mosby, Inc; 2005;
  2. Alexandros A. Drosos. Relapsing polychondritis. Orphanet Encyclopedia. October 2004; http://www.orpha.net/data/patho/GB/uk-RP.pdf. Accessed 12/19/2011.
  3. Compton N, Buckner JH, Harp KI, Raugi GJ. Polychondritis. Medscape Reference. January 19, 2012; http://emedicine.medscape.com/article/331475-overview. Accessed 6/10/2013.