Relapsing Polychondritis

Relapsing Polychondritis

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Relapsing Polychondritis is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • chronic atrophic polychondritis
  • generalized or systemic chondromalocia
  • Meyenburg-Altherr-Uehlinger syndrome
  • relapsing perichondritis
  • von Meyenburg disease

Disorder Subdivisions

  • None

General Discussion

Relapsing polychondritis is a rare degenerative disease characterized by recurrent inflammation of the cartilage in the body. Deterioration of the cartilage may affect any site of the body where cartilage is present. Ears, larynx and trachea may become "floppy," and the bridge of the nose can collapse into a "saddlenose" shape. The aortic heart valve may also be affected.

Symptoms

Symptoms of relapsing polychondritis usually begins with the sudden onset of pain, tenderness and swelling of the cartilage of one or both ears. This inflammation may spread to the fleshy portion of the outer ear causing it to narrow. Attacks may last several days to weeks before subsiding. Middle ear inflammation can cause obstruction of the eustachian tube. Recurrent attacks may lead to hearing loss.



Nasal Chondritis may be marked by cartilage collapse at the bridge of the nose resulting in a saddlenose deformity, nasal stuffiness or fullness and crusting.



Inflammation of both large and small joints can occur. Classic symptoms of pain and swelling are similar to those of arthritis.



Involvement of the cartilage of the larynx and bronchial tubes may cause breathing and speech difficulties.



Heart valve abnormalities may occur.



Relapsing polychondritis may also cause kidney inflammation and dysfunction.

Causes

The exact cause of relapsing polychondritis is not known. It is thought to be an autoimmune disease. Autoimmune disorders are caused when the body's natural defenses against "foreign" or invading organisms (e.g., antibodies) begin to attack healthy tissue for unknown reasons. Some cases may be linked to abnormal reactions by blood cells (serum antibodies), to a thyroid protein (thyroglobulin), organ wall (parietal) cells, adrenal cells, or thyroid. Symptoms of relapsing polychondritis may arise when autoantibodies attack human cartilage.



Some researchers believe that relapsing relapsing polychondritis may be caused by an immunologic sensitivity to type II collagen, a normal substance found in skin and connective tissue.

Affected Populations

Relapsing polychondritis affects males and females in equal numbers. Symptoms usually begin between forty and sixty years of age.

Standard Therapies

Treatment of relapsing polychondritis usually involves the administration of corticosteroid drugs (e.g., prednisone), aspirin and non-steroidal anti-inflammatory compounds such as dapsone and/or colchicine. In extreme cases, drugs that suppress the immune system such as cyclophosphamide, 6-mercaptopurine and azathioprine may be recommended. In the most severe cases replacement of heart valves or the insertion of a breathing tube (tracheotomy) for collapsed airways may be necessary.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government website.



For information about clinical trials being conducted at the National Institutes of Health (NIH) in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com



Some cases of relapsing polychondritis may go into remission after use of the immune suppressing drug cyclosporine-A. However, more research is necessary to determine complete safety and effectiveness of this treatment.



In the medical literature, there is a report of a child with relapsing polychondritis whose symptoms improved after treatment with type II collagen (CII). More research is necessary to determine the effectiveness and long-term safety of this potential treatment for relapsing polychondritis.

References

TEXTBOOKS

Stein, JH, ed. Internal Medicine. 4th ed. St. Louis, MO:Mosby-Year Book, Inc.; 1994:2406, 2465.



Kelley WN, et al., eds. Textbook of Rheumatology. 4th ed. Philadelphia, PA: W.B. Saunders Company; 1993:1400-09.



JOURNAL ARTICLES

Navarro MJ, et al. Amelioration of relapsing polychondritis in a child treated with oral collagen. Am J Med Sci. 2002;324:101-3.



Letko E, et al. Relapsing polychondritis: a clinical review. Semin Arthritis Rheum. 2002;31:384-95.



Balsa-Criado A, et al. Cardiac involvement in relapsing polychondritis. Int J Cardiol. 1987;14:381-83.



Michet CJ, et al. Relapsing polychondritis. Survival and predictive role of early disease manifestations. Ann Intern Med. 1986;104:74-78.



Krell WS, et al. Pulmonary function in relapsing polychondritis. Am Rev Respir Dis. 1986;133:1120-23.



INTERNET

Compton N. Polychondritis. Emedicine. http://emedicine.medscape.com/article/331475-overview. Updated January 19, 2012. Accessed February 15, 2012.



Genetic and Rare Diseases Information Center (GARD). Relapsing polychondritis. http://www.rarediseases.info.nih.gov/GARD/Disease.aspx?PageID=4&diseaseID=7417. Accessed February 15, 2012.

Resources

Arthritis Foundation

1330 West Peachtree Street, Suite 100

Atlanta, GA 30309

USA

Tel: (404)872-7100

Tel: (800)283-7800

Email: arthritisfoundation@arthritis.org

Internet: http://www.arthritis.org



NIH/National Institute of Arthritis and Musculoskeletal and Skin Diseases

Information Clearinghouse

One AMS Circle

Bethesda, MD 20892-3675

USA

Tel: (301)495-4484

Fax: (301)718-6366

Tel: (877)226-4267

TDD: (301)565-2966

Email: NIAMSinfo@mail.nih.gov

Internet: http://www.niams.nih.gov/



Rheumatology Outpatient Clinic at Beth Israel Hospital

Division of Rheumotology

Department of Medicine

West Campus, Lowry Medical Office Building, 4B

110 Francis Street

330 Brookline Avenue

Boston, MA 02215

Tel: (617)632-8658

Internet: http://www.bidmc.org/CentersandDepartments/Departments/Medicine/Divisions/Rheumatology.aspx



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



Madisons Foundation

PO Box 241956

Los Angeles, CA 90024

Tel: (310)264-0826

Fax: (310)264-4766

Email: getinfo@madisonsfoundation.org

Internet: http://www.madisonsfoundation.org



Autoimmune Information Network, Inc.

PO Box 4121

Brick, NJ 08723

Fax: (732)543-7285

Email: autoimmunehelp@aol.com



European Society for Immunodeficiencies

1-3 rue de Chantepoulet

Geneva, CH 1211

Switzerland

Tel: 410229080484

Fax: 41229069140

Email: esid@kenes.com

Internet: http://www.esid.org



For a Complete Report

This is an abstract of a report from the National Organization for Rare Disorders, Inc.® (NORD). Cigna members can access the complete report by logging into myCigna.com. For non-Cigna members, a copy of the complete report can be obtained for a small fee by visiting the NORD website. The complete report contains additional information including symptoms, causes, affected population, related disorders, standard and investigational treatments (if available), and references from medical literature. For a full-text version of this topic, see http://www.rarediseases.org/search/rdblist.html.

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