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- $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.
-
-