What
Is Behçet's Disease?
The disease was first described in 1937 by
Dr. Helusi Behçet, a professor of dermatology
in Istanbul. Behçet'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 Behçet's
Disease?
Behçet'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. Behçet's
disease tends to develop in people in their
twenties or thirties, but people of all ages
can develop it.
What Causes Behçet's
Disease?
The exact cause of Behçet'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.
Behçet'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 Behçet's disease.
What Are the Symptoms
of Behçet's Disease?
Behçet'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 Behçet'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
Behçet'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 Behçet's disease,
physicians must observe symptoms to make an
accurate diagnosis. The four most common symptoms
of Behçet'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 Behçet'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 Behçet'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 Behçet'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 Behçet's disease.
Arthritis causes pain, swelling, and stiffness
in the joints, especially the knees, ankles,
wrists, and elbows. Arthritis that results
from Behçet's disease usually lasts
a few weeks and does not cause permanent
damage to the joints.
- Vasculitis-This is
an inflammation of blood vessels and can
cause small strokes, severe skin ulcers
(called pathergy), mouth or genital ulcers,
of rupture of large blood vessels in the
lung, chest, or abdomen.
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In addition
to mouth and genital sores, eye inflammation,
and arthritis, Behçet's disease may
cause other skin problems, blood clots, and
inflammation in the central nervous system
and digestive organs.
Skin
Problems
Behçet'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 Behçet'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 Behçet's disease may develop a
bump where the doctor pricked the skin. Doctors
disagree about the usefulness of a pathergy
test because Behçet'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 Behçet'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
Behçet'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. Behçet'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 Behçet'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 Behçet's Disease Diagnosed?
Diagnosing Behçet's disease is very
difficult because no specific test confirms
it. Less than half of the patients initially
thought to have Behçet'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 Behçet'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 Behçet'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.
How
Is Behçet's Disease Treated?
Although there is no cure for Behçet'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 Behçet'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 Behçet'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 Behçet'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 Behçet'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 Behçet'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 Behçet'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 Behçet's disease keep
their joints strong and flexible.
What
Is the Prognosis for a Person With Behçet's
Disease?
Most people with Behçet'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 Behçet's disease.
What
Are Researchers Trying To Learn About Behçet's
Disease?
Researchers are exploring possible genetic,
bacterial, and viral causes of Behçet'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
Behçet's disease. They are particularly
interested in whether Streptococcus, the bacterium
that causes strep throat, is associated with
the disease. Many people with Behçet'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 Behçet'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. |
