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Last updated:
September 19, 2018
Years published: 1988, 1989, 1991, 1998, 2002, 2007, 2009, 2012, 2015, 2018
NORD gratefully acknowledges Naveed Younis, MD, Consultant Physician, Department of Medicine, University Hospital of South Manchester, Wythenshawe Hospital, United Kingdom, for assistance in the preparation of this report.
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 of relapsing polychondritis usually begin 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 saddle nose 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.
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.
Relapsing polychondritis affects males and females in equal numbers. Symptoms usually begin between forty and sixty years of age.
Treatment
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.
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
Some current clinical trials also are posted on the following page on the NORD website:
https://rarediseases.org/living-with-a-rare-disease/find-clinical-trials/
For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com
For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/
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.
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. Medscape. Updated: Sep 25, 2017. http://emedicine.medscape.com/article/331475-overview Accessed Sept. 13, 2018.
NORD strives to open new assistance programs as funding allows. If we don’t have a program for you now, please continue to check back with us.
NORD and MedicAlert Foundation have teamed up on a new program to provide protection to rare disease patients in emergency situations.
Learn more https://rarediseases.org/patient-assistance-programs/medicalert-assistance-program/Ensuring that patients and caregivers are armed with the tools they need to live their best lives while managing their rare condition is a vital part of NORD’s mission.
Learn more https://rarediseases.org/patient-assistance-programs/rare-disease-educational-support/This first-of-its-kind assistance program is designed for caregivers of a child or adult diagnosed with a rare disorder.
Learn more https://rarediseases.org/patient-assistance-programs/caregiver-respite/
