(Amicale de Behcet en Belgique)
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(Amicale de Behcet en Belgique)
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Links Medicine Net What are symptoms of Behcet's syndrome? How is Behcet's syndrome diagnosed? What is the treatment of Behcet's syndrome? Terms Related To Behcet's Syndrome: What is Behcet's
syndrome? The cause of Behcet's syndrome is not known. The disease is more frequent and severe in patients from the Eastern Mediterranean and Asia than those of European descent. Both inherited (genetic) and environmental factors, such as microbe infections, are suspected to be factors that contribute to the development of Behcet's syndrome. Behcet's syndrome has not been felt to be contagious. What are symptoms
of Behcet's syndrome? The mouth and genital ulcers are generally
painful and recur in crops. They range in size from a few millimeters to 20
millimeters in diameter. The mouth ulcers occur on the gums, tongue, and inner
lining of the mouth. The genital ulcers occur on the scrotum and penis of males
and vulva of women and can leave scars. Inflammation of the eye, which can involve
the front of the eye (uvea) causing uveitis, or the back of the eye (retina)
causing retinitis, can lead to blindness. Symptoms of eye inflammation include
pain, blurred vision, tearing, redness, and pain when looking at bright lights.
It is very important for patients to have this sensitive area monitored by an
eye specialist (ophthalmologist). If the arteries become inflamed (arteritis)
in patients with Behcet's syndrome, it can lead to death of the tissues whose
oxygen supply depends on these vessels. This could cause a stroke if affecting
the brain, belly pain if affecting the bowel, etc. When veins become inflamed (phlebitis),
the inflammation can involve large veins that develop blood clots which can
loosen to cause pulmonary embolism. Symptoms of inflammation of the brain or
tissue that covers the brain (meninges) include headaches, neck stiffness, and
is often associated with fever. Inflammation of the brain (encephalitis) and/or
the meninges (meningitis) can cause damage to nervous tissue and lead to
weakness or impaired function of portions of the body. This can result in
confusion and coma. Typically these features occur later in the disease course,
years after the diagnosis. Joint inflammation (arthritis) can lead to
swelling, stiffness, warmth, pain, and tenderness of joints in patients with
Behcet syndrome. This occurs in about half of patients with Behcet's syndrome at
some time during their lives. Knees, wrists, ankles, and elbows are the most
common joints affected. The skin of patients with Behcet's syndrome
can develop areas of inflammation which spontaneously appear as raised, tender,
reddish nodules (erythema nodosum), typically on the front of the legs. Some
patients with Behcet's syndrome develop a peculiar red or blistery skin reaction
in places where they have been pierced by blood-drawing needles (see pathergy
test in diagnosis section below). Recent research has found that acne occurs
more frequently in patients with Behcet's syndrome that also have arthritis as a
manifestation. Ulcerations can occur at any location in the stomach, large or small bowel in patients with Behcet's disease.
How is Behcet's
syndrome diagnosed? Tests, such as skin biopsy, lumbar puncture, MRI scan of the brain, and bowel tests are considered based upon symptoms that are present.
What is the
treatment of Behcet's syndrome? Steroid (cortisone) gels, pastes (such as
KENOLOG in ORABASE) and creams can be helpful for the mouth and genital ulcers.
Colchicine can also minimize recurrent ulcerations. Joint inflammation can require
non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral
steroids. Colchicine and oral and injectible cortisone are used for inflammation
involving the joints, eyes, skin, and brain. Sulfasalazine has been effective in
some patients for arthritis. Bowel disease is treated with oral steroids and sulfasalazine (AZULFADINE). Severe disease of the arteries, eyes, and
brain can be difficult to treat and require powerful medications that suppress
the immune system called immunosuppressive agents. Immunosuppressive agents used
for severe Behcet's syndrome include chlorambucil (LEUKERAN), azathioprine (IMURAN),
and cyclophosphamide (CYTOXAN). Cyclosporine has been used for resistant disease.
Recent studies suggest that thalidomide may
be of benefit for certain patients with Behcet's syndrome in treating and
preventing ulcerations of the mouth and genitals. Side effects of thalidomide
include promoting abnormal development of fetal growth, nerve injury (neuropathy),
and hypersedation. Recent research further suggests that
patients who have had heart valve replacement because of severe damage as a
result of inflammation can benefit by immune suppression with azathioprine and
prednisone after operation. Trials are currently underway evaluating
interferon alpha for the treatment of eye disease in patients with Behcet's
syndrome.
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