Relapsing Polychondritis



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Relapsing Polychondritis is an uncommon, chronic disorder of the cartilage that is characterized by recurrent episodes of inflammation of the cartilage of various tissues of the body. Tissues containing cartilage that can become inflamed include the ears, nose, joints, spine, and windpipe. The eyes, heart, and blood vessels, which have a biochemical makeup similar to that of cartilage, can also be affected.

Symptoms:

  • Pain
  • Redness
  • Swelling
  • Tenderness in one or both ears, the nose, throat, joints, and/or eyes.
  • Fever
  • Fatigue
  • Weight loss

  • Typically, this disease causes sudden pain in the inflamed tissue at the onset of the disease.

    Inflammation of the ears and nose can cause deformity from weakened cartilage. Impaired hearing, balance, and nausea can be caused by inner ear inflammation.

    Inflammation of the windpipe or trachea can lead to throat pain, hoarseness, and breathing difficulty. This is a potentially dangerous area of inflammation in patients with relapsing polychondritis, which can require assisted breathing methods when severe.

    Joint inflammation can cause pain, swelling, and stiffness of the joints, including of the hands, knees, ankles, wrists, and feet.

    Eye inflammation can be mild or severe and can damage vision. Cataracts can be caused by the inflammation or from the cortisone used to treat relapsing polychondritis.

    Other tissues that can develop inflammation include the aorta which can lead to aneurysm or aortic valve weakness, tissues in or around the heart, the skin, and the nerves from the brain.

    Causes:
    The cause is unknown. It is suspected that this condition is caused by an immune system disorder in which the body's immunity system is misguided. This results in inflammation that is directed at various tissues of the body.

    Treatment:
    For patients with more mild disease, nonsteroidal anti-inflammatory drugs (NSAIDs), including ibuprofen (Motrin), naproxen (Naprosyn), and others, can be helpful to control the inflammation. Usually, however, cortisone-related medications (steroids such as prednisone and prednisolone) are required. High-dose steroids are frequently necessary initially, especially when the eyes or breathing airways are involved. Moreover, most patient require steroids for long-term use.

    Methotrexate (Rheumatrex, Trexall) has shown promise as a treatment for relapsing polychondritis in combination with steroids as well as a maintenance treatment. Studies have demonstrated that methotrexate can help reduce the steroid requirements.

    Other medications that have been tried in small numbers of patients with some reports of success include cyclophosphamide (Cytoxan), dapsone, azathioprine (Imuran), penicillamine (Depen, Cuprimine), cyclosporine, and combinations of these drugs with steroids.


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    ...less medical jargon in a 'Quick Glance' format!