Relapsing polychondritis

What causes 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.

Last updated on 05-01-20

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:

In some cases, a biopsy of affected tissue may be necessary to support the diagnosis.

Last updated on 05-01-20

Is relapsing polychondritis inherited?

Relapsing polychondritis (RP) is not passed through families in a clear-cut fashion. Most people with relapsing polychondritis do not have affected relatives.

Like many other autoimmune conditions, RP is likely a multifactorial condition which is associated with the effects of multiple genes in combination with lifestyle and environmental factors. In general, having a first degree relative (for example a parent, child, or sibling) with an autoimmune condition may increase your personal risk for developing an autoimmune condition. Unfortunately, no specific risk estimates are available for relapsing polychondritis.

Last updated on 05-01-20

What is the long-term outlook for people with relapsing polychondritis?

The long-term outlook (prognosis) for people with relapsing polychondritis (RP) varies from person to person. In general, RP is a chronic and progressive (worsening overtime) condition. Some form of disability is common in the later stages of RP; these may include visual impairment, hearing loss, vestibular dysfunction, and/or cardiopulmonary (heart and lung) disease.

Severe cases of RP can be life-threatening. Respiratory complications (windpipe collapse and infections) are the most common cause of death followed by cardiovascular (heart and blood vessel) involvement.

In recent years, improvements have been made in the outcomes of patients with RP. Survival rates have increased from 70% after 5 years, to 94% after 8 years and even 91% after 10 years in a recent study.

Last updated on 05-01-20

How might relapsing polychondritis be treated?

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.

Last updated on 05-01-20

Patient Registry

Benaroya Research Institute

The Translational Research Program at the Virginia Mason Benaroya Research Institute maintains a Patient Registry for relapsing polychondritis. To learn more, contact the study coordinator Sylvia Posso by calling 206-342-6975.

Last updated on 04-27-20

Name: Arthritis Foundation 1355 Peachtree St. NE 6th Floor
Atlanta, GA, 30309, United States
Phone: +1-404-872-7100 Toll Free: 1-844-571-HELP (4357) Url: https://www.arthritis.org
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: aarda@aarda.org Url: https://www.aarda.org/
Name: Relapsing Polychondritis Awareness and Support Foundation, Inc RPASF, Inc. 1202 Lexington Avenue, Box 112
New York, NY, 10028,
Email: admin@polychondritis.org Url: https://polychondritis.org/
Name: Canadian Society for Relapsing Polychondritis Canada Url: https://polychondritis.ca/

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