Relapsing Polychondritis

Relapsing polychondritis facts

• Relapsing polychondritis is an uncommon, chronic disorder of the cartilage.
• Relapsing polychondritis is characterized by recurrent episodes of painful inflammation.
• Relapsing polychondritis can involve all types of cartilage.
• Typical cartilage tissues affected include the ears, nose, and joints.
• There is no one specific test for diagnosing relapsing polychondritis.
• Treatment often involves cortisone-related medications.
• The course of symptoms for patients is often unpredictable.

What is relapsing polychondritis?

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. Chondritis means inflammation of cartilage. Tissues containing cartilage that can become inflamed include the ears, nose, joints, spine, and windpipe (trachea). The eyes, heart, and blood vessels, which have a biochemical makeup similar to that of cartilage, can also be affected. Relapsing polychondritis is sometimes called the red ear syndrome.

What causes relapsing polychondritis?

The cause of relapsing polychondritis is unknown. It is suspected that this condition is caused by "autoimmunity." Autoimmunity is characterized by a misdirected immune system. This results in inflammation in various tissues of the body.

What are symptoms and signs of relapsing polychondritis?

Typically, relapsing polychondritis causes sudden pain in the inflamed tissue at the onset of the disease. Common symptoms are pain, redness, swelling, and tenderness in one or both ears, the nose, throat, joints, and/or eyes. The lobe of the ear is not involved. Fever, fatigue, and weight loss often develop.
Inflammation of the ears and nose can cause deformity (saddle nose deformity and floppy ears) 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 and can require assisted breathing methods when severe.
Joint inflammation (arthritis) 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 (see below).
Other tissues that can develop inflammation include the aorta (which can lead to aneurysm or aortic valve weakness), tissues in or around the heart (myocarditis and pericarditis), the skin (vasculitis), and the nerves from the brain (cranial nerve palsies).

How is relapsing polychondritis diagnosed?

Relapsing polychondritis is diagnosed when the doctor recognizes the classic pattern of cartilage involvement during the history and physical examination. The symptoms described above can suggest the disease.
There is no one specific test for diagnosing relapsing polychondritis. Blood tests that indicate inflammation, such as an elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and others, are often abnormal when the disease is active.
If tissue cartilage is biopsied, the involved cartilage will demonstrate nonspecific signs of inflammation.

Can relapsing polychondritis be associated with other diseases?

Yes. The doctor will be interested in determining whether or not signs of the following diseases are present along with relapsing polychondritis: vasculitis, Wegener's granulomatosis, systemic lupus erythematosus, ankylosing spondylitis, Reiter's disease, psoriatic arthritis, rheumatoid arthritis, Behcet's disease, Churg-Strauss syndrome, polyarteritis nodosa, myelodysplasia, and others.

What medications are used to treat relapsing polychondritis?

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, anti-tumor necrosis factor (TNF) biologic medications (adalimumab [Humira], infliximab [Remicade]), and combinations of these drugs with steroids.

What is the long-term outlook (prognosis) for patients with relapsing polychondritis?

The course of symptoms for patients with relapsing polychondritis is often unpredictable.
Repeated bouts of inflammation of cartilage from relapsing polychondritis frequently leads to permanent destruction of the involved tissues and results in disability. Destruction of nose and ear cartilage results in deformity and can impair breathing when the trachea is affected.
Relapsing polychondritis is potentially dangerous and even life threatening, depending on the tissues involved. Inflammation of the cartilage of the windpipe (trachea), heart, aorta, and other blood vessels can be fatal. For some patients, however, the disease is much more limited and mild. Close monitoring of symptoms with a qualified doctor is recommended for optimal results.

REFERENCE:

Klippel, J.H., et al. Primer on the Rheumatic Diseases. New York: Springer, 2008.

Medical Author:

William C. Shiel Jr., MD, FACP, FACR

Medical Editor:

Catherine Burt Driver, MD

Source:Medicinenet.com.