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    Rare Disease Causes Inflammation of Cartilage Throughout Body

Rare Disease Causes Inflammation of Cartilage Throughout Body

March 16, 2012

Dear Mayo Clinic:
I was just diagnosed with relapsing polychondritis. What has been your experience with this disease?


Relapsing polychondritis is a rare disease that causes inflammation of cartilage throughout the body, especially in the ears, nose, eyes and throat. Symptoms of this disease come and go. The severity of symptoms, and how often they appear, varies quite a bit from person to person. Although no cure currently exists for relapsing polychondritis, it often can be effectively controlled with medication.

The specific cause of relapsing polychrondritis is not known. The underlying trigger for the disease could be related to a person's genetic makeup, and triggered by environmental factors. But, whatever the cause, relapsing polychrondritis appears to be an autoimmune disorder. In these disorders, the immune system mistakenly targets healthy organs and tissue. In this case, it attacks the body's cartilage.

Cartilage is the firm, flexible tissue that cushions your joints and gives shape to other parts of your body, such as your nose, ears and windpipe. In relapsing polychondritis, cartilage becomes inflamed, causing redness, swelling, irritation, discomfort and pain in the affected areas. The inflammation of this disease may cause your windpipe, or trachea, to narrow, resulting in hoarseness, shortness of breath and wheezing. In addition, relapsing polychrondritis can cause complications in the heart, lungs, blood vessels, kidneys and arthritis affecting both small and large joints. Arthritis is also a common complication.

Because symptoms come and go, and are not specific to the disease, diagnosing relapsing polychrondritis can be difficult. For example, the first symptoms many people experience are redness, pain and increased warmth of the ears. This can often be mistaken for an insect bite or an infection, leading to an incorrect diagnosis and a prescription for antibiotics.

Because the severity and frequency of symptoms can be so different from one person to another, once the disease is diagnosed, you may need quite a few appointments with your doctor. These visits can help your doctor gauge the aggressiveness of your relapsing polychrondritis, determine what type of treatment you need, and watch for complications.

If the disease is mild, a nonsteroidal anti-inflammatory drug may suffice in relieving symptoms. In more severe cases, medications that suppress the immune system may be necessary. Some people's bodies respond very quickly to treatment for relapsing polychrondritis, while others are quite resistant. So being able to work closely with your doctor to have treatment tailored to your situation is key to effectively managing this disease.

Even after you and your doctor have established a treatment plan, you will still need regular follow-up appointments. Everyone with this disease, even those with mild forms, should be watched closely because serious complications can develop quickly.

For people who were treated for this disease in the 1970s and 1980s, the long-term outlook was not very good. That has changed significantly over the last two decades. Even though there is still no cure, the prognosis for people with relapsing polychrondritis is now quite good. That is due in large part to the much wider range of drugs we have to choose from to help control its symptoms. Today, with close monitoring and prompt, individualized treatment, most people who have relapsing polychrondritis can lead normal lives.

— Harvinder Luthra, M.D., Rheumatology, Mayo Clinic, Rochester, Minn.