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What Is RP?

Relapsing Polychondritis: A rare, debilitating and potentially fatal connective tissue disorder. Commonly displayed by inflammation of the cartilage and proteoglycan rich structures.

– Marcela A. Ferrada, M.D, Clinical Consultant Center for Human Immunology, National Institutes of Health

What Is Relapsing Polychondritis?

Relapsing polychondritis (RP) is a rare (and in some cases fatal) autoimmune disease characterized by recurrent widespread inflammation of different body areas – in particular, the tissue around the joints called cartilage. Cartilage is a firm tissue, softer than bone, found in several areas of the body such as the joints, nose, and ear.  Is also found in the trachea and airways.

Autoimmune disease is an inflammatory condition where the body attacks its own healthy cells, believing them to be foreign. The exact mechanism of RP is unknown, but different studies have found elevation of certain antibodies against collagen in some patients with RP, suggesting that there is an autoimmune component.

RP can affect many parts of the body including the ears, nose, airways, joints, eyes, kidneys, and blood vessels.

Because RP is a “relapsing” condition, it can improve for a period of time and then worsen, marking it difficult to diagnose.

How Is Relapsing Polychondritis diagnosed?

The diagnosis of RP is purely clinical meaning there is no blood test to identify the condition.  To make the diagnosis, the patient needs to have 3 or more of the following symptoms: recurrent inflammation of one or both ears, inflammation in more than one joint, inflammation of the bridge of the nose, inflammation of the eyes, inflammation of the trachea or airways, inflammation of the ear usually manifested by hearing loss, tinnitus (buzzing or ringing in the ears) and/or dizziness.  In some instances, the patients may have a biopsy (a sample of body tissue that is examined under the microscope) to help with the diagnosis, but it is not necessary if the patient already exhibits sufficient symptoms, and in some instances, performing a biopsy on already-inflamed tissue can make the disease worse.

Many patients with RP have a delay in diagnosis. Patients can have symptoms for many years before they are diagnosed.

Who Gets Relapsing Polychondritis?

RP is an uncommon disease that affects approximately 3-5 individuals per million people. RP affects both men and women. It can affect individuals of all ages – it has been described in childhood and beginning as late as age 80, but it most frequently occurs midlife.

What Should I Do if I Think I Have RP?

You should talk to your doctor about being referred to a rheumatologist, a specialist in treating conditions such as RP, rheumatoid arthritis, and lupus.  The rheumatologist may need to perform different tests to rule out other conditions.

Learn More About the Symptoms of Relapsing Polychondritis

Frequently Asked Questions

It was initially thought that all patients with RP would have severe health problems due to the disease. It is now known that there is a great deal of variation in the severity of the disease from person to person. Many individuals may have mild symptoms, which recur infrequently, while others may have persistent problems that become debilitating or life-threatening. At this time it is not known how to predict how the disease will affect any specific individual.

Treatment for relapsing polychondritis is approached in a manner similar to other autoimmune diseases. A team approach is very helpful in treating this disease as it can involve many organs. Initial treatment usually includes prednisone and further medical treatment should be done with the supervision of a rheumatologist and may include other medications. Treatment of eye disease should be done in consultation with an opthalmologist, and those individuals with respiratory symptoms may need to be followed by an otolaryngologist or pulmonologist.

Although RP is rare, medical research is being conducted in the US and Japan to try to expand understanding of the disease. The current data about the mechanism of the disease, as well as the best treatment approach, is very limited. In order to improve patient outcomes with RP, research is necessary to gain a better understanding of the disease, develop a classification of disease severity, and to develop better treatment options.

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