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Relapsing Polychondritis

What is Relapsing Polychondritis?

Relapsing Polychondritis (RP) is a rare disease in which the body’s cartilage undergoes recurrent inflammation. Cartilage is a firm tissue that is found in many places in the body, including between bone joints, at the end of the ribs, in the ear and nose as well as in the airway tubes of the lungs and the valve in the heart. RP is a chronic, progressive disease that can cause joint and facial deformity and be life threatening if the lungs or heart are affected. The exact cause is unknown and treatment typically involves anti-inflammatory drugs.

 

 

 

Relapsing Polychondritis (RP) is a rare disease in which the body’s cartilage undergoes recurrent inflammation. Cartilage is a firm tissue that is found in many places in the body, including between bone joints, at the end of the ribs, in the ear and nose as well as in the airway tubes of the lungs and the valve in the heart. RP is a chronic, progressive disease that can cause joint and facial deformity and be life threatening if the lungs or heart are affected. The exact cause is unknown and treatment typically involves anti-inflammatory drugs.

 

 

Acknowledgement of Relapsing Polychondritis has not been added yet.

The annual incidence for relapsing polychondritis is about 3.5 cases per million. RP affects men and women equally and symptoms typically manifest most often in people in their 50s and 60s.

Synonyms for Relapsing Polychondritis has not been added yet.

              The exact cause of relapsing polychondritis is unknown, but
researchers believe it may be an autoimmune disease. In autoimmune disorders,
the body’s own immune system not only attacks invading foreign bacteria or
viruses, it attacks its own healthy tissue, and in the case of RP, it may
attack cartilage.

              Some researchers believe that RP may be due to a sensitivity to Type
II Collagen, which is a naturally occurring substance found in connective
tissue. Researchers do know that RP is not an inherited disease and is not
passed on through family members. However, there is some evidence to suggest
that individuals with RP are twice as likely to carry a gene called HLA-DR4. HLA-DR4
does not cause RP, but this gene, along with other unknown genetic factors as
well as the environment, may play a contributing role to RP.

The onset of the disease begins with sudden pain and swelling in the cartilage. The attacks can last for several days or weeks and can lead to the deterioration of the cartilage. Nonspecific symptoms such as fever, weight loss or fatigue may also be present. The characteristics of the disease and the severity of the symptoms vary from person to person and depend on where the inflamed cartilage is located.

  • Ears – The ears are most commonly affected in RP. The outer part of the ear loses its firmness and becomes floppy. Inflammation of the inner ear is possible, as well as hearing impairment. Twenty percent of individuals with RP have this symptom at the onset and 90% will develop it at some point.
  • Joints- The second area most prone to attacks are the joints, with symptoms similar to arthritis. Thirty-three percent of individuals with RP have this symptom at the onset and 80% will develop it at some point.
  • Nose- Inflammation of the nose cartilage may lead to stuffiness, nose bleeds, crusting, excessive mucus, nose deformity and potentially a loss of smell. Less than one-fifth of individuals with RP have this symptom at the onset and 65% will develop it at some point.
  • Airways- The trachea, larynx and bronchi are airway structures that are made up of cartilage and inflammation can result in coughing, wheezing, recurrent pulmonary infection and difficulty breathing. 10% of individuals with RP have this symptom at the onset and 50% will develop it at some point.
  • Eyes- Inflammation can affect the eyes, leading to red, bulging eyes and possibly a loss of vision. This symptom is very rare at the onset, but 60% of RP individuals will develop it.
  • Less common areas affected by RP: The heart, kidneys, nervous system, and gastrointestinal tract.

A correct diagnosis can only be made by a medical professional. There is no test available that is specific to identifying relapsing polychondritis, so a diagnosis is dependent on the presence of three or more of the following characteristics: inflammation of cartilage in both ears, arthritis of joints, inflammation of cartilage in the nose, eye inflammation, inflammation of cartilage in the throat, or vestibular dysfunction.

A biopsy of the affected cartilage may also need to be performed to confirm the diagnosis.

Diagnostic tests of Relapsing Polychondritis has not been added yet

Treatment for relapsing polychondritis is focused on relieving pain and preserving the structure of the affected cartilage. Most often, treatment involves the administration of anti-inflammatory drugs such as corticosteroids with prednisone to decrease the severity of the flare ups. Other anti-inflammatory drugs such as aspirin, dapsone and colchicine can also be administered. In more severe cases, there can be the administration of immunosuppressants, such as cyclophosphamide and azathioprine.

For
individuals with life threatening RP, replacement of heart valves or insertion
of breathing tubes may be needed.

The prognosis for individuals with relapsing polychondritis varies. The disease is chronic and progressive, which means the longer the individual has it, the worse it gets. There is usually some sort of disability associated with RP, such as visual or hearing impairment. RP can be life threatening if there is respiratory collapse or cardiovascular damage. The prognosis has improved recently; the five year survival rate was seventy percent and the ten year survival rate was fifty five percent. Now, the eight year survival rate is ninety four percent and the ten year survival rate is 91%.

Tips or Suggestions of Relapsing Polychondritis has not been added yet.
Discussion Created by geoergettecb8
Last updated 30 Jul 2014, 12:17 PM

Posted by geoergettecb8
30 Jul 2014, 12:17 PM

I am interested in communicating with rjones, if possible. I am in northern VA and am in poor health and frustrated with treatment by my physicians. If you are willing to speak via email or just on this forum it would be greatly appreciated. Thank you. Georgette

Treatment success Created by kssaari
Last updated 9 Apr 2014, 10:44 AM

Posted by geoergettecb8
9 Apr 2014, 10:44 AM

Hi, I also was also diagnosed last fall, finally, I knew I had it for several years and had to search for the right doctor to properly diagnose me. I'm undergoing treatment with methotrexate and low dose steroids but haven't been having much success. In the beginning the treatment made me feel like a completely new person, my old self, I guess. Lately symptoms are returning and new symptoms have emerged. I am seeing my doctor today, and will post later. Thank you for sharing. Georgette

Posted by Sisvh1
8 Apr 2014, 11:24 PM

There can be success in putting this disease in remission with the right meds. It is a trial and error solution not ideal but all we have. There is an extensive forum on Facebook. Just type in Relapsing Polychondritis. I have had RP since 2007 but there are people on the Facebook site that have had it for much longer. I hope this helps.

Posted by kssaari
4 Jan 2014, 03:37 AM

I have recently been diagnosed with relapsing polychondritis. Has anyone had some success in treating this? I am from the Midwest and was wondering what is available out there.

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