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$Unique_ID{BRK03520}
$Pretitle{}
$Title{Behcet's Syndrome}
$Subject{Behcet's Syndrome Adamantiades-Behcet's syndrome Gilbert's (W.)
Syndrome Halushi-Behcet's Syndrome oculobuccogenital Syndrome Touraine's
Aphthosis Triple Symptom Complex of Behcet Reiter Syndrome Stevens-Johnson
Syndrome Ulcerative Colitis}
$Volume{}
$Log{}

Copyright (C) 1986, 1987, 1988, 1989, 1990, 1992 National Organization
for Rare Disorders, Inc.

100:
Behcet's Syndrome

** IMPORTANT **
It is possible that the main title of the article (Behcet's Syndrome) is
not the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Adamantiades-Behcet's syndrome
     Gilbert's (W.) Syndrome
     Halushi-Behcet's Syndrome
     oculobuccogenital Syndrome
     Touraine's Aphthosis
     Triple Symptom Complex of Behcet

Information can be found on the following diseases in the Related
Disorders section of this report.

     Reiter Syndrome
     Stevens-Johnson Syndrome
     Ulcerative Colitis

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.


Behcet's Syndrome is a relapsing multi-system inflammatory disease.  The
most common manifestation is a triad of oral and genital ulcers, and
inflammation of the eyes.  The joints, blood vessels, central nervous system,
and gastrointestinal tract may also be involved.  Attacks last a week to a
month, and recur spontaneously.  Some symptoms can appear as late as several
years after onset of the disease.  Onset is usually between age 20 and 30;
twice as many men as women are affected.  The disease is most common in the
Middle East and Japan.

Symptoms

Changes in the microscopic appearance of the blood vessels occur in all
involved organs of patients with Behcet's Syndrome.  Symptoms come and go,
and do not necessarily coincide with each other.  During acute attacks, there
may be fever.

The earliest symptoms usually consist of painful sores, or ulcers, in the
mouth, lasting several days or weeks.  Subsequently, similar, painful ulcers
may appear on the genitalia (scrotum, occasionally the penis; labia, vulva,
and vaginal wall) and perineum (the skin surrounding the genitalia and anus).
Like the oral lesions, these may last up to a month.  Different parts of one
or both eyes may become involved, causing pain, irritation, and visual
disturbances.  Inflammation of the eyes is recurrent; usually lesions
disappear, but sometimes cataracts, glaucoma, and blindness may develop.

About half the patients have arthritis before, during, or after the
appearance of other symptoms.  Large joints, such as elbows, ankles, and
knees, are involved more often than small ones.  The arthritis may be mild,
asymptomatic, or may become chronic.  Only very seldom is there permanent
damage to the joints.

Blood vessel involvement sometimes takes the form of thrombophlebitis
(inflammation of a vein associated with the formation of a thrombus, or blood
clot).  Thrombophlebitis occurs in less than 20% of patients with Behcet
syndrome.  It is recurrent, and the inflammation may move from one vein to
another.  Both superficial and deep veins usually in the legs become
involved; occasionally the large veins feeding directly into the heart, are
affected.  Rarely, pulmonary embolism develops.

In about a quarter of the patients, the central nervous system becomes
involved; this can be early or late in the course of disease.  Manifestations
include meningoencephalitis (inflammation of the brain and its covering
tissues), benign increased intracranial pressure (high fluid pressure inside
the skull), lesions in the brainstem and/or spinal cord, paralysis of muscles
served by large nerves known as cranial nerves, and psychosis.  Central
nervous system involvement tends to fluctuate and may be life threatening.

Gastrointestinal symptoms range from vague abdominal discomfort to a
syndrome similar to ulcerative colitis or regional enteritis, with nausea,
vomiting, abdominal bloating, ulcers, malabsorption, pain, etc.  Discrete
ulcer in the colon or ileum can bleed, perforate or cause pain.

Skin symptoms include various rashes, bumps, etc.  The legs and arms are
affected most often.  These skin conditions are called folliculitis and
erythema nodosum.

Complications may include blindness, paralysis, and obstruction of the
vena cava.  Fatalities usually result only from nervous system,
gastrointestinal, and vascular involvement.

Causes

The causes of Behcet's syndrome are not understood.  Genetic predisposition,
autoimmune mechanisms, and viral infection may all play a role.  In 1989
Japan's Ministry of Health & Welfare announced that they discovered a new
strain of bacteria in the teeth of 70% of patients with Behcet's Syndrome
which may be related to the cause of the disease.  The bacterium is similar
to Streptococcci bacteria, but classification of the organism is not yet
possible.

Affected Population

Twice as many men as women in Middle Eastern And Asian countries are affected
with Behcet's Syndrome, usually between the ages of 20 and 30 years.  The
disorder occurs far more frequently in the Middle East and Japan than
elsewhere.  The incidence in Japan according to one study is 1 in 10,000
individuals, or 15,000 people living in Japan.  It is the leading cause of
blindness in these areas.  In the United States and Britain, women are
affected more often than men; however, the disease is less severe.  It
affects approximately 15,000 persons in the U.S.

Related Disorders

Reiter Syndrome is characterized by inflammation of the joints, urethra, and
conjunctiva of the eye, and by lesions of the skin and mucous surfaces.
Arthritis is the main problem.  Symptoms do not necessarily appear
simultaneously; they may alternate, and there may be spontaneous remissions
and reoccurrences.  The syndrome rarely disables its victims.  It appears to
result from an abnormal immune response and association with exposure
(usually sexual) to an infectious agent.  It primarily affects men between
the ages of twenty and forty years.  (For more information, choose "Reiter"
as your search term in the Rare Disease Database.)

Stevens-Johnson Syndrome is a severe form of Erythema Multiforme
characterized by blistery lesions on the mucous membranes of the mouth,
throat, anogenital region, eyelids, and corneal lining (conjunctiva) of the
eye.  (For more information, choose "Stevens-Johnson" as your search term in
the Rare Disease Database.

Ulcerative Colitis is a non-specific inflammatory disease of the bowel
characterized by chronic ulceration of the large intestine.  The chief
characteristic of this disorder is bloody diarrhea.  This disease of unknown
cause generally begins in the rectosigmoid area.  It may involve only the
left side of the colon or may eventually attack most of the large bowel
simultaneously.  The disease is usually chronic, with repeated periods of
exacerbation and remission.  (For more information on this disorder, choose
"Ulcerative Colitis" as your search term in the Rare Disease Database.)

Therapies:  Standard

Spontaneous remission is fortunately common for patients with Behcet's
Syndrome.  Therapy is symptomatic.  Locally applied corticosteroids can
control ocular and oral symptoms.  Xylocaine mouthwashes often relieve oral
pain.  Systemic corticosteroids benefit patients with severe eye
inflammations or central nervous system involvement.  Corticosteroids do not,
however, prevent recurrences of symptoms.

Therapies:  Investigational

Immunosuppressive drugs such as azathioprine, cyclophosphamide, cyclosporine,
and chlorambucil have helped some patients with Behcet's Syndrome.  Transfer
factor, a substance from white blood cells that can provide the ability to
mount a specific kind of immunological response, prepared from healthy
donors, has also helped some patients.

The orphan drug thalidomide is being tested as a treatment for Behcet's
Syndrome.  This drug should not be taken by pregnant women because it can
cause severe birth defects.  Physicians wishing to test thalidomide as a
treatment for this disorder may contact:

     Andrulis Research Corp.
     4600 East West Highway, Suite 900
     Bethesda, MD  20814

Thalidomide is available in England under special license from Penn
Pharmaceuticals of Tredegar, South Wales.

The immunosuppressant drug, Cyclosporine, (Sandimmune) is being tested as
a treatment for the oral ulcers, skin lesions, and eye manifestations of
Behcet's Syndrome.  A study of ninety-six patients in Japan (published in The
Lancet, May 20, 1989), indicates the treatment may be beneficial, but more
research is needed to determine the long-term safety and effectiveness of
cyclosporine as a treatment for Behcet's Syndrome.

Cyclosporine is also being used to treat Uveitis, an eye complication of
this disorder.  Studies in the United States, Japan, and Israel indicate that
Cyclosporine may diminish or prevent recurrent attacks of Uveitis in Behcet
patients.

The immunosuppressant drug Imuran (azathioprine) has controlled the
progression of eye disease in clinical tests on people with Behcet's
Syndrome.  More research is needed to determine the long-term safety and
effectiveness of Imuran on this disorder.

This disease entry is based upon medical information available through
February 1992.  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 Behcet's Syndrome, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     American Behcet's Association, Inc.
     P.O. Box 54063
     Minneapolis, MN  55454-0063
     [phone]
     (800)-7BEHCETS

     Arthritis Foundation
     1314 Spring Street, NW
     Atlanta, GA  30309
     [phone]

     J.D. Duffy, M.D.
     Mayo Clinic Behcet's Clinic
     Dept. of Rheumatology
     Rochester, MN  55905

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al: eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1256.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W. B. Saunders Co., 1988.  Pp. 2048-9, 1609, 2026.

CYCLOSPORINE IN BEHCET'S DISEASE RESISTANT TO CONVENTIONAL THERAPY, Laura
E. Caspers-Velu, et al., Annals of Ophthalmology (issue 1989).

TREATMENT OF INTRAOCULAR INFLAMMATORY DISEASE WITH CYCLOSPORINE A.
Nussenblatt, Robert B, et al.; The Lancet, 1983.