Learn About Relapsing Polychondritis

What is the definition of Relapsing Polychondritis?
Relapsing polychondritis (RP) is characterized by recurrent swelling and inflammation of cartilage and other tissues throughout the body. Cartilage is a tough but flexible tissue that covers the ends of bones at a joint and gives shape and support to other parts of the body. Symptoms of RP include swelling of the cartilage of the ear, nose, and joints.  Other parts of the body that may be involved are the airways (trachea), costal (rib) cartilage, eyes, heart, vascular (veins) system, skin, kidney, and nervous system. The signs and symptoms vary from person to person depending on which parts of the body are affected. The exact underlying cause of RP is unknown. There are thought to be genetic and other unknown factors involved. RP often occurs along with autoimmune conditions. Diagnosis is based on the symptoms and clinical examination. Other more common conditions may need to be excluded before RP can be diagnosed. The primary goals of treatment for people with RP are to relieve present symptoms and to preserve the structure of the affected cartilage.
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What are the alternative names for Relapsing Polychondritis?
  • Chronic atrophic polychondritis
  • Recurrent polychondritis
  • Polychondropathia
What are the causes of Relapsing Polychondritis?
The exact underlying cause of relapsing polychondritis (RP) is unknown. However, scientists suspect that it is an autoimmune condition. It it thought that RP occurs when the body's immune system mistakenly attacks its own cartilage and other tissues. In general, autoimmune conditions are complex traits that are associated with the effects of multiple genes in combination with lifestyle and environmental factors. There is also evidence to suggest that some people may be born with a genetic susceptibility to RP. Studies have found that people with RP are roughly twice as likely as those without this condition to carry a certain genetic allele called HLA-DR4. "HLA" stands for human leukocyte antigen, which is an important part of our immune system and plays a role in resistance and predisposition (risk) to disease. However, HLA genes are not solely responsible for specific diseases but instead may simply contribute along with other genetic or environmental factors to disease risk. Thus, many people with HLA-DR4 will never develop RP.
What are the symptoms of Relapsing Polychondritis?
The following list includes the most common signs and symptoms in people with relapsing polychondritis (RP). These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition The features of the condition and the severity of symptoms vary significantly from person to person, but may include:
  • Pain and swelling of the ear
  • Damage to the outer part of the ear
  • Swelling of the inner ear
  • Dizziness, hearing loss, and/or nausea
  • Joint pain
  • Swelling of the voice box (larynx)
  • Narrowing and blockage of the trachea (tracheal stenosis)
  • Coughing, wheezing, or hoarseness
  • Swelling of the outer parts of the eye (episcleritis, uveitis and/or scleritis).
  • Nasal cartilage inflammation and damage
Less commonly, RP may affect the heart, kidneys, nervous system, gastrointestinal tract, and/or vascular (veins) system. Nonspecific symptoms such as fever, weight loss, malaise, and fatigue may also be present. Symptoms usually begin in adulthood between the ages of 20 and 60, but RP has been diagnosed in children as well.  In approximately one third of affected people, RP is associated with other medical problems. Conditions reportedly associated with RP include hematological diseases (including Hodgkin's lymphoma and myelodysplastic syndromes); gastrointestinal disorders (including Crohn's disease and ulcerative colitis); endocrine diseases (including diabetes mellitus type 1 and thyroid disorders) and others. Episodes of RP may last a few days or weeks and typically resolve with or without treatment. However, it is generally progressive, and many people have persistent symptoms in between flares. The most serious symptoms involve the airways and heart.
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What are the current treatments for Relapsing Polychondritis?
The primary goals of treatment for people with relapsing polychondritis (RP) are to relieve present symptoms and to preserve the structure of the affected cartilage. The main treatment for RP is corticosteroid therapy with prednisone to decrease the severity, frequency and duration of relapses. Higher doses are generally given during flares, while lower doses can typically be prescribed during periods of remission. Other medications reported to control symptoms include dapsone, colchicine, azathioprine, methotrexate, cyclophosphamide, hydroxychloroquine, cyclosporine and infliximab. People who develop severe heart or respiratory complications may require surgery. More detailed information about the management of RP is available on Medscape Reference's Web site and can be viewed by clicking here.
Who are the top Relapsing Polychondritis Local Doctors?
Elite
Highly rated in
15
conditions

University Of Tokyo

Tokyo, JP 

Jun Shimizu is in Tokyo, Japan. Shimizu is rated as an Elite expert by MediFind in the treatment of Relapsing Polychondritis. He is also highly rated in 15 other conditions, according to our data. His top areas of expertise are Relapsing Polychondritis, Myositis, Behcet Disease, and Kawasaki Disease.

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Highly rated in
12
conditions
Rheumatology

MedStar Georgetown University Hospital

Washington, DC 

Kaitlin Quinn is a Rheumatologist in Washington, Washington, D.c.. Dr. Quinn has been practicing medicine for over 11 years and is rated as an Elite doctor by MediFind in the treatment of Relapsing Polychondritis. She is also highly rated in 12 other conditions, according to our data. Her top areas of expertise are Takayasu Arteritis, Relapsing Polychondritis, Vasculitis, and Giant Cell Arteritis. She is licensed to treat patients in District of Columbia.

 
 
 
 
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Elite
Highly rated in
4
conditions

St. Marianna University School Of Medicine

Kawasaki, JP 

Noboru Suzuki is in Kawasaki, Japan. Suzuki is rated as an Elite expert by MediFind in the treatment of Relapsing Polychondritis. He is also highly rated in 4 other conditions, according to our data. His top areas of expertise are Relapsing Polychondritis, Aniridia, Kawasaki Disease, and Behcet Disease.

How is Relapsing Polychondritis diagnosed?
There are no tests available that are specific for relapsing polychondritis (RP). A diagnosis is, therefore, generally based on the presence of characteristic signs and symptoms. For example, people may be diagnosed as having RP if they have three or more of the following features:
  • Inflammation of the cartilage of both ears
  • Seronegative (negative for rheumatoid factor) polyarthritis (arthritis that involves 5 or more joints simultaneously)
  • Inflammation of the cartilage of the nose
  • Eye inflammation (conjunctivitis, episcleritis, scleritis, and/or uveitis)
  • Inflammation of the cartilage of the airway
  • Vestibular dysfunction (i.e. vertigo, hearing loss, tinnitus)
In some cases, a biopsy of affected tissue may be necessary to support the diagnosis.
What are the latest Relapsing Polychondritis Clinical Trials?
Studies of the Natural History, Pathogenesis, and Outcome of Idiopathic Systemic Vasculitis
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Longitudinal Study for Relapsing Polychondritis
What are the Latest Advances for Relapsing Polychondritis?
Subacute encephalopathy associated with relapsing polychondritis. Report of one case.
When You "Can't See" a Case of Relapsing Polychondritis.
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Unusual Presentation of Relapsing Polychondritis in a Patient with Human Immunodeficiency Virus and Reactive Arthritis.