What Is Behcet's Disease?
The disease was first described in 1937 by Dr. Helusi
Behcet, a professor of dermatology in Istanbul. Behcet's disease is now
recognized as a chronic condition that causes sores or ulcers in the
mouth and on the genitals, and inflammation in parts of the eye. In some
people, the disease also results in arthritis (swollen, painful, stiff
joints) and inflammation of the digestive tract, brain, and spinal
cord.
Who Gets Behcet's Disease?
Behcet's disease is common in the Middle East, Asia, and
Japan, but rare in the United States. In Middle Eastern and Asian
countries, the disease affects more men than women. In the United
States, the opposite is true. Behcet's disease tends to develop in
people in their twenties or thirties, but people of all ages can develop
it.
What Causes Behcet's Disease?
The exact cause of Behcet's disease is unknown. Most of
the symptoms are caused by inflammation of the blood vessels,
particularly veins. Inflammation is the body’s characteristic reaction
to injury or disease and is marked by four signs: swelling, redness,
heat, and pain. Doctors think that an autoimmune reaction may cause
blood vessels to become inflamed, but they do not know what triggers
this reaction. In an autoimmune reaction, the immune system mistakenly
attacks and harms the body’s own tissues. Under normal conditions, the
immune system protects the body from diseases and infections by killing
harmful foreign substances, such as germs, that enter the body.
Behcet's disease is not contagious and does not spread
from one person to another. Researchers think that two factors are
probably important in its development. First, it is believed that
abnormalities of the immune system make some people susceptible to the
disease. Researchers think that this problem may be inherited; that is,
it may be due to one or more specific genes. Second, something in the
environment, possibly a bacterium or virus, might trigger or activate
the disease in susceptible people. Researchers have found that people
who have frequent strep infections (caused by Streptococcus
bacteria) are more likely to develop Behcet's disease.
What Are the Symptoms of Behcet's
Disease?
Behcet's disease affects each person differently. Some
people have only mild symptoms, such as skin sores or ulcers in the
mouth or on the genitals. Others have more severe disease, such as
meningitis or inflammation of the membranes that cover the brain and
spinal cord. Meningitis can cause fever, a stiff neck, and headaches.
More severe symptoms usually appear months or years after a person
notices the first signs of Behcet's disease. Symptoms can last for a
long time or can come and go in a few weeks. Typically, symptoms appear,
disappear, then reappear. The times when a person is having symptoms are
called flares. To help the doctor diagnose Behcet's disease and monitor
its course, patients may want to keep a record of the symptoms that
occur and when they occur. Because many conditions mimic Behcet's
disease, physicians must observe symptoms to make an accurate diagnosis.
The four most common symptoms of Behcet's disease are mouth sores,
genital sores, inflammation of parts of the eye, and arthritis.
- Mouth sores Mouth sores (known as oral aphthosis and
aphthous stomatitis) affect almost all patients with Behcet's disease.
They are often the first symptom that a person notices and may occur
long before any other symptoms appear. The sores usually have a red
border and several may appear at the same time. They can be painful
and make eating difficult. Mouth sores go away in 10 to 14 days but
often come back. Small sores usually heal without scarring, but larger
ones may scar.
- Genital sores Affecting more that half of all people with
Behcet's disease, most genital sores appear on the scrotum in men and
vulva in women. The sores look similar to mouth sores and may be
painful. After several outbreaks, they may cause scarring.
- Uveitis (yoo.vee.EYE.tis) Inflammation of the middle part
of the eye (the uvea), including the iris, occurs in more than half of
all people with Behcet's disease. This symptom is more common among
men than women and typically begins within 2 years of the first
symptoms. Eye inflammation can cause blurred vision and, rarely, pain
and redness. Because partial loss of vision or blindness can result if
the eye frequently becomes inflamed, patients should report these
symptoms to their doctor immediately.
- Arthritis Inflammation of the joints occurs in more than
half of all patients with Behcet's disease. Arthritis causes pain,
swelling, and stiffness in the joints, especially the knees, ankles,
wrists, and elbows. Arthritis that results from Behcet's disease
usually lasts a few weeks and does not cause permanent damage to the
joints.
In addition to mouth and genital sores, eye inflammation,
and arthritis, Behcet's disease may cause other skin problems, blood
clots, and inflammation in the central nervous system and digestive
organs.
Skin Problems
Behcet's disease causes various skin sores that look like
red bumps on a black-and-blue mark. The sores are red, raised, and
typically appear on the legs and upper torso. In some people, sores or
lesions may appear when the skin is scratched or pricked. When doctors
suspect that a person may have Behcet's disease, they may perform a test
called pathergy in which they prick the skin with a small needle: 1 to 2
days after the test, people with Behcet's disease may develop a bump
where the doctor pricked the skin. Doctors disagree about the usefulness
of a pathergy test because Behcet's patients in the United States rarely
have a skin reaction. However, more than half of the patients in Middle
Eastern countries and Japan do have a reaction.
Blood Clots
About 10 percent of patients with Behcet's disease have
blood clots resulting from inflammation in the veins (thrombophlebitis),
usually in the legs. Symptoms include pain and tenderness in the
affected area, which may also be swollen and warm. Because
thrombophlebitis can have severe complications, people should report
symptoms to their doctor immediately. A few patients may experience
artery problems such as aneurysms (a stretching or expanding of a
weakened blood vessel).
Central Nervous System
Behcet's disease affects the central nervous system in
about 10 percent of all patients with the disease. The central nervous
system includes the brain and spinal cord and helps the body to
coordinate movements and process information. Behcet's disease can cause
meningoencephalitis inflammation of the brain and the thin membrane
that covers and protects it. People with meningoencephalitis may have
fever, headache, stiff neck, and difficulty coordinating movement, and
should report any of these symptoms to their doctor immediately. If this
condition is left untreated, a stroke can result.
Digestive Tract
Only rarely does Behcet's disease cause inflammation and
ulceration (sores) in the digestive tract and lead to stomach pain,
diarrhea, constipation, and vomiting. Because these symptoms are very
similar to symptoms of other diseases of the digestive tract, such as a
peptic ulcer, ulcerative colitis, and especially Crohn's disease,
careful evaluation is essential.
How Is Behcet's Disease Diagnosed?
Diagnosing Behcet's disease is very difficult because no
specific test confirms it. Less than half of the patients initially
thought to have Behcet's disease actually have it. The doctor must
examine a patient with symptoms and rule out other conditions with
similar symptoms. Because it may take several months or even years for
all the common symptoms to appear, the diagnosis may not be made for a
long time. A patient may even visit several different kinds of doctors
before the diagnosis is made.
These symptoms are key to diagnosing Behcet's disease:
- Mouth sores at least three times in 12 months
- Any two of the following symptoms: recurring genital sores, eye
inflammation with loss of vision, skin lesions, or positive pathergy
(skin prick test).
Besides finding these signs, the doctor must rule out
other conditions with similar symptoms, such as Crohn's disease and
Reiter's syndrome. The doctor may also recommend that the patient see an
eye specialist to identify possible complications related to eye
inflammation.
What Kind of Doctor Treats a
Patient With Behcet's Disease?
Because the disease affects different parts of the body, a
patient will probably see several different doctors. It may be helpful
to both the doctors and the patient for one doctor to manage the
complete treatment plan. This doctor can coordinate treatment and
monitor any side effects from the various medications the patient
takes.
A rheumatologist (a doctor specializing in arthritis)
often manages the patient's overall treatment and treats joint disease.
The following specialists treat other symptoms that affect different
body systems:
- Gynecologist treats enital sores in women.
- Urologist treats genital sores in men.
- Dermatologist treats genital sores in men, and skin and
mucous membrane problems.
- Ophthalmologist treats eye inflammation.
- Gastroenterologist treats digestive tract symptoms.
- Neurologist treats central nervous system symptoms.
How Is Behcet's Disease Treated?
Although there is no cure for Behcet's disease, people can
usually control their symptoms with proper medication, rest, and
exercise. Treatment goals are to reduce discomfort and prevent serious
complications such as disability from arthritis or blindness. The type
of medicine and the length of treatment depend on the person's symptoms
and their severity.
It is likely that a combination of treatments will be
needed to relieve specific symptoms. Patients should tell each of their
doctors about all of the medicines they are taking so that the doctors
can coordinate treatment.
Topical Medicine
Topical medicine is applied directly on the sores to
relieve pain and discomfort. For example, doctors prescribe rinses to
treat mouth sores. Creams are used to treat skin and genital sores. The
medicine usually contains corticosteroids, which reduce inflammation, or
an anesthetic, which relieves pain.
Oral Medicine
Doctors also prescribe medicines taken by mouth to reduce
inflammation throughout the body, suppress the overactive immune system,
and relieve symptoms. Doctors may prescribe one or more of the medicines
described below to treat the various symptoms of Behcet's disease.
- Corticosteroids Prednisone is a corticosteroid prescribed
to reduce pain and swelling throughout the body in people with severe
joint pain and inflammation, skin sores, eye disease, or central
nervous system symptoms. Patients must carefully follow the doctor's
instructions about when to take prednisone and how much to take. It is
also important not to stop taking the medicine suddenly because it
alters the body's production of the natural corticosteroid hormones.
Long-term use of prednisone can have side effects such as
osteoporosis, weight gain, delayed wound healing, persistent
heartburn, and elevated blood pressure. However, these side effects
are rare when prednisone is taken at low doses for a short time. It is
important that patients see their doctor regularly to monitor possible
side effects.
- Immunosuppressive drugs Medicines (including
corticosteriods) that help control an overactive immune system, such
as is the case in people with Behcet's disease, reduce inflammation
throughout the body and can lessen the number of flares. Doctors may
use immunosuppressive drugs when a person has eye disease or central
nervous system involvement. These medicines are very strong and can
have serious side effects. Patients must see their doctor regularly
for blood tests to detect and monitor side effects.
Depending on the person's specific symptoms, doctors may
use one or more of the following immunosuppressive drugs:
- Azathioprine Most commonly prescribed for people with organ
transplants because it suppresses the immune system, azathioprine is
now used to treat uveitis and central nervous system involvement in
Behcet's disease. This medicine can upset the stomach and may reduce
the production of new blood cells by the bone marrow.
- Chlorambucil Doctors use chlorambucil to treat uveitis and
meningoencephalitis. People taking chlorambucil must see their doctor
frequently because it can have serious side effects, such as permanent
sterility and cancers of the blood. Patients need regular blood tests
to monitor blood counts of white cells and platelets.
- Cyclosporine Like azathioprine, doctors prescribe this
medicine for people with organ transplants. When used by patients with
Behcet's disease, cyclosporine reduces uveitis and central nervous
system involvement. To reduce the risk of side effects, such as kidney
and liver disease, the doctor can adjust the dose. Patients must tell
their doctor if they take any other medicines, because some affect the
way the body uses cyclosporine.
- Colchicine Commonly used to treat gout, which is a form of
arthritis, colchicine reduces inflammation throughout the body. The
medicine is sometimes used to treat eye inflammation and skin symptoms
in patients with Behcet's disease. Common side effects of colchicine
include nausea, vomiting, and diarrhea. The doctor can decrease the
dose to relieve these side effects.
If these medicines do not reduce symptoms, doctors may use
other drugs such as cyclophosphamide and methotrexate. Cyclophosphamide
is similar to chlorambucil. Methotrexate, which is also used to treat
various kinds of cancer as well as rheumatoid arthritis, can relieve
Behcet's symptoms because it suppresses the immune system and reduces
inflammation throughout the body.
Rest and Exercise
Although rest is important during flares, doctors usually
recommend moderate exercise, such as swimming or walking, when the
symptoms have improved or disappeared. Exercise can help people with
Behcet's disease keep their joints strong and flexible.
What Is the Prognosis for a Person
With Behcet's Disease?
Most people with Behcet's disease can lead normal lives
and control their symptoms with proper medicine, rest, and exercise.
Doctors can use many medicines to relieve pain, treat symptoms, and
prevent complications. When treatment is effective, flares usually
become less frequent after 1 or 2 years. Many patients eventually enter
a period of remission. In some people, however, treatment does not
relieve symptoms, and gradually more serious symptoms such as eye
disease may occur. Serious symptoms may appear months or years after the
first signs of Behcet's disease.
What Are Researchers Trying To
Learn About Behcet's Disease?
Researchers are exploring possible genetic, bacterial, and
viral causes of Behcet's disease, as well as improved drug treatment.
Researchers hope to identify genes that increase a person's chance of
developing the disease. Studying these genes and how they work may lead
to a new understanding of the disease and possibly new treatments.
Researchers are also investigating factors in the
environment, such as a bacterium or virus, that could trigger Behcet's
disease. They are particularly interested in whether Streptococcus, the
bacterium that causes strep throat, is associated with the disease. Many
people with Behcet's disease have had several strep infections. In
addition, researchers suspect that herpes virus type I, a virus that
causes cold sores, may be associated with the disease.
Finally, researchers are identifying other medicines to
better treat Behcet's disease. Thalidomide, for example, appears
effective in treating severe mouth sores, but its use is experimental
and very limited. Thalidomide is not used in women of childbearing age
because it causes severe birth defects.
Keeping on Top of Your Condition
Keeping in tune with your disease or condition not only makes treatment less intimidating but also increases its chance of success, and has been shown to lower a patients risk of complications. As well, as an informed patient, you are better able to discuss your condition and treatment options with your physician.
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