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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.