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 (trachea). The eyes, heart, and blood vessels, which have a biochemical makeup similar to that of cartilage, can also be affected.

Alternative Names of Polychondritis are: Relapsing polychondritis.

Complications of Polychondritis

The list of complications that have been mentioned in various sources for Polychondritis includes:

• Hearing loss in recurring cases
• Death
• Aortic valve weakness
• Aneurysm

Causes of Polychondritis

The cause of relapsing polychondritis is unknown. It is suspected that this condition is caused by an immune system disorder (autoimmunity) in which the body's immunity system (which normally fights off invaders of the body, particularly infections) is misguided. This results in inflammation that is directed at various tissues of the body.

Signs & Symptoms of 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. 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, which 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.
• 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).

Diagnosis of Polychondritis

• 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 to the doctor.
• 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.

Treatments of 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 patients 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.

Prevention of Polychondritis

Because the cause is unknown, there is no way to prevent polychondritis. Complications can be prevented with appropriate medical attention. For example, if there is severe inflammation of the windpipe (trachea), a tracheal stent, a small tube that keeps the airway open, can be inserted until medications are able to control the disease.

When to seek Medical Advice

If you have the symptoms of polychondritis, especially if you have difficulty breathing, contact your health care professional.