Polychondritis

What Is It?

Polychondritis, also called relapsing polychondritis, is a rare disease in which cartilage in many areas of the body becomes inflamed. The disease most commonly affects the ears, nose and the airways of the lungs. The cause is not known, and it occurs most often in people in their 50s or 60s. One theory is that polychondritis might be an autoimmune disease, in which the immune system attacks the body rather than foreign invaders such as viruses. In polychondritis, it's possible that a triggering event, perhaps an infection, sets off a reaction by the immune system, which unleashes an attack on the body's cartilage. Some people may have a genetic makeup that makes them more prone to this. The disease does not seem to run in families. It sometimes appears in people who have other disease, such as rheumatoid arthritis, vasculitis (inflammation of blood vessels) and systemic lupus erythematosus (SLE or lupus).

Symptoms

Polychondritis is a systemic (body-wide) illness. Common symptoms include:

  • Fatigue or malaise

  • Fever

  • Red, swollen, painful (inflamed) ears, hearing loss, dizziness

  • Ears that are "floppy," that is, they are softer than normal, limp or droopy

  • Inflammation over the bridge of the nose, nasal congestion

  • Arthritis

  • Shortness of breath, cough, stridor (high-pitched sound during breathing)

  • More rarely: eye inflammation, leaky heart valves, kidney disease, neurological problems, rash

Diagnosis

The diagnosis may be delayed, since early symptoms can be similar to symptoms of other conditions that are much more common than polychondritis. A biopsy of an inflamed area is required to confirm the diagnosis. In a biopsy, a small piece of tissue is removed and examined under a microscope. The biopsy also is helpful to rule out other causes of symptoms such as infection, especially tuberculosis, syphilis, leprosy and fungal disease. Sometimes, the biopsy is not conclusive, or the doctor is certain enough of the diagnosis that a biopsy does not have to be done. No single blood test will give a definite diagnosis, including tests for antibodies to cartilage. Imaging studies, including a computed tomography (CT) scan of the lungs and other tests, especially pulmonary-function tests, may be performed to help determine the extent of disease.

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