$Unique_ID{BRK04117} $Pretitle{} $Title{Polychondritis} $Subject{Polychondritis Chronic Atrophic Polychondritis Relapsing Polychondritis Rheumatoid Arthritis Osteoarthritis Behcet Syndrome} $Volume{} $Log{} Copyright (C) 1988, 1989, 1991 National Organization for Rare Disorders, Inc. 561: Polychondritis ** IMPORTANT ** It is possible that the main title of the article (Polychondritis) is not the name you expected. Please check the synonym list to find the alternate names and disorder subdivisions covered by this article. Synonyms Chronic Atrophic Polychondritis Relapsing Polychondritis Relapsing Perichondritis Generalized or Systemic Chondromalocia von Meyenburg Disease Meyenburg-Altherr-Uehlinger Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Rheumatoid Arthritis Osteoarthritis Behcet Syndrome General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. 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 be involved as well. Symptoms Symptoms of 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. Polychondritis may also cause kidney inflammation and dysfunction. Causes The exact cause of Polychondritis is not known. It is thought to be an autoimmune disease. Autoimmune disorders are caused when the body's natural defenses (antibodies) against invading organisms begin to attack perfectly healthy tissue. 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 polychondritis may arise when autoantibodies attack human cartilage. Some researchers believe that relapsing Polychondritis may be caused by an immunologic sensitivity to type II collagen, a normal substance found in skin and connective tissue. Affected Population Polychondritis affects males and females in equal numbers. Symptoms usually begin between forty and sixty years of age. Related Disorders Symptoms of the following disorders can be similar to those of polychondritis. Comparisons may be useful for a differential diagnosis: Rheumatoid Arthritis is a disease of unknown origin which may have a relationship to autoimmune processes. This disorder is characterized by lack of appetite (anorexia), tiredness, painful and deformed joints, early morning stiffness chiefly in the hands, knees, feet, jaw, and spine. Once affected, a patient's joints remain painful or uncomfortable for weeks, months, or even years. Osteoarthritis is a degenerative joint disease of unknown origin characterized by loss of cartilage, deformities of bones with joints, and extra cartilage and bone growth at the joint margins with subsequent bony enlargement. Osteoarthritis develops when cartilage repair does not keep pace with degeneration. It may occur as a result of trauma to the bony or underlying joint disease. Behcet Syndrome is an inflammatory disorder affecting a number of organs. The most constant symptom is of oral and genital ulcers. Eye and joint inflammation, similar to Polychondritis, occurs. Blood vessels, the central nervous system, and the gastrointestinal tract may also be involved. Attacks last a week to a month, and can recur spontaneously. Some symptoms can appear as late as several years after onset of the disease which usually occurs between age 20 and 30. Twice as many men as women are affected. The disease is most common in the Middle East and Japan. For more information on the above disorder, choose "Behcet" as your search term in the Rare Disease Database. Therapies: Standard Treatment of polychondritis usually involves the administration of corticosteroid drugs, aspirin and non-steroidal anti-inflammatory compounds. 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. Therapies: Investigational Some cases of 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. This disease entry is based upon medical information available through February 1991. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Polychondritis, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 Polychondritis & Rheumatoid Arthritis Clinic David Trentham, M.D., Chief, Division of Rheumatology Beth Israel Hospital 330 Brookline Ave. Boston, MA 02215 (617) 735-2560 The National Arthritis and Musculoskeletal and Skin Diseases (NIAMS) Information Clearinghouse Box AMS Bethesda, MD 20892 (301) 495-4484 Arthritis Foundation 1314 Spring St, NW Atlanta, Ga. 30309 (404) 872-7100 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 631, 664, 1311. TEXTBOOK OF RHEUMATOLOGY, Kelly, 1988. Chapter 84, Polychondritis, Jerome H. Herman, Pp. 2-31. CARDIAC INVOLVEMENT IN RELAPSING POLYCHONDRITIS: A. Balsa-Criado, et al.; Int J Cardiol (March, 1987, issue 14 (3)). Pp. 381-383. RELAPSING POLYCHONDRITIS. SURVIVAL AND PREDICTIVE ROLE OF EARLY DISEASE MANIFESTATIONS: C.J. Michet, et al.; Ann Intern Med (January, 1986, issue 104 (1)). Pp. 74-78. PULMONARY FUNCTION IN RELAPSING POLYCHONDRITIS: W. S. Krell, et al.; Am Rev Respir Dis (June, 1986, issue 133 (6)). Pp. 1120-1123.