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There is no cure for
Behçet's disease. Treatment typically focuses on reducing discomfort
and preventing serious complications. Corticosteroids and
other medications that suppress the immune system may be prescribed
to treat inflammation. Behçet's is a chronic disease that recurs.
However, patients may have periods of time when symptoms go away
temporarily (remission). The severity of the disease varies from
patient to patient. Some patients may live somewhat normal lives,
but others may become blind or severely disabled.
Behçets disease
affects different parts of the body, therefore, a patient probably
will 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 the treatments and
monitor any side effects from the various medications that the
patient takes.
A rheumatologist (a
doctor specializing in arthritis and other inflammatory disorders)
often manages a patient's treatment and treats joint disease. The
following specialists also treat other symptoms that affect the
different body systems:
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Gynecologist-treats
genital sores in women
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Urologist-treats
genital sores in men
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Dermatologist-treats genital sores in men and women, and skin
and mucous membrane problems
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Ophthalmologist-treats eye inflammation
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Gastroenterologist-treats digestive tract symptoms
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Hematologist-treats
disorders of the blood
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Neurologist-treats central nervous system symptoms
Although there is no
cure for Behçet's disease, people usually can control symptoms with
proper medication, rest, exercise, and a healthy lifestyle. The goal
of treatment is 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, gels, or ointments. Creams are
used to treat skin and genital sores. The medicine usually contains
corticosteroids (which reduce inflammation), other anti-inflammatory
drugs, 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. The treatment of Behçet's
syndrome depends on the severity and the location of its
manifestations in an individual patient.
Steroid (cortisone) gels, pastes (such as Kenalog in Orabase) and
creams can be helpful for the mouth and genital ulcers.
Colchicine
can also minimize
recurrent ulcerations. Mouth and genital ulcers healed and were
reported at a national meeting of the American College of
Rheumatology as less frequent in 9 or 12 patients who were treated
with Trental (pentoxifylline).
Trental also seemed to maintain the healed ulcers for up to the 29
months of the study. The effectiveness of Trental, the researchers
said, seemed to be enhanced by the combination with colchicine in
some patients.
Joint
inflammation can require non-steroidal anti-inflammatory drugs (such
as
ibuprofen and
others) or oral steroids. Colchicine and oral and injectable
cortisone are used for inflammation involving the joints, eyes,
skin, and brain. Sulfasalazine has
been effective in some patients for arthritis.
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Corticosteroids - Prednisone is a corticosteroid prescribed
to reduce pain and inflammation throughout the body for people
with severe joint pain, 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 also is important not to stop taking the medicine
suddenly, because the medicine alters the body's production of
the natural corticosteroid hormones. Long-term use of prednisone
can have side effects such as osteoporosis (a disease that leads
to bone fragility), 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.
Corticosteroids are useful in early stages of disease and for
acute severe flares. They are of limited use for long-term
management of central nervous system and serious eye
complications.
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Immunosuppressive drugs - These medicines (in addition to
corticosteriods) help control an overactive immune system, which
occurs in Behçet's disease, and reduce inflammation throughout
the body, and can lessen the number of disease 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.
Doctors may use one
or more of the following drugs depending on the person's specific
symptoms.
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Azathioprine (Imuran) -
classified as an immunosuppressant
medication. Azathioprine is used to suppress the immune system
in patients who have had kidney transplants. Although its exact
mechanism of action in
rheumatoid arthritis
is not known, its effect in suppressing
the immune system appears to decrease the activity of this
illness. Most commonly prescribed for people with organ
transplants because it suppresses the immune system,
azathioprine is now used for people with Behçet's disease to
treat uveitis and other uncontrolled disease manifestations.
This medicine can upset the stomach and may reduce production of
new blood cells by the bone marrow.
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Chlorambucil -
Doctors may use these drugs to treat uveitis and
meningoencephalitis. People taking either agent must see their
doctor frequently because either can have serious side effects,
such as permanent sterility and cancers of the blood. Patients
have regular blood tests to monitor blood counts of white cells
and platelets.
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Colchicine -
Commonly used to treat gout, which is a form of arthritis,
colchicine reduces inflammation throughout the body. The
medicine sometimes is used to treat arthritis, mucous membrane,
and skin symptoms in patients with Behçet's disease. A research
study in Turkey suggested that the medication works best for
males with the disorder. Common side effects of colchicine
include nausea, vomiting, and diarrhea. The doctor can decrease
the dose to relieve these side effects.
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Cyclophosphamide - drug that is used primarily
for treating several types of
cancer.
In order to work, cyclophosphamide first is converted by the
liver into two chemicals, acrolein and phosphoramide. Acrolein
and phosphoramide are the active compounds, and they slow the
growth of cancer cells by interfering with the actions of
deoxyribonucleic acid (DNA) within the cancerous cells. It is,
therefore, referred to as a cytotoxic drug. Unfortunately,
normal cells also are affected, and this results in serious side
effects. Cytoxan also suppresses the immune system and is also
referred to as immunosuppressive.
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Cyclosporine -
Like azathioprine, doctors prescribe this medicine for people
with organ transplants. When used by patients with Behçet's
disease, cyclosporine reduces uveitis and uncontrolled disease
in other organs. 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 medicines affect the way the body uses
cyclosporine.
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Enbrel (Etanetcept) -
Etanercept is an injectable drug that
blocks tumor necrosis factor alpha (TNF alpha) and is used for
treating rheumatoid
arthritis,
ankylosing spondylitis, and psoriatic arthritis. TNF alpha is a
protein that the body produces during the inflammatory response,
the body's reaction to injury. TNF alpha promotes the
inflammation and its associated fever and signs (pain,
tenderness, and swelling) in several inflammatory conditions
including rheumatoid arthritis
and
ankylosing spondylitis. Etanercept is a synthetic (man-made)
protein that binds to TNF alpha. It thereby acts like a sponge
to remove most of the TNF alpha molecules from the joints and
blood. This prevents TNF alpha from promoting inflammation and
the fever, pain, tenderness and swelling of joints in patients
with rheumatoid or psoriatic arthritis and ankylosing
spondylitis. Etanercept reduces the signs and symptoms of
rheumatoid arthritis, the arthritis of psoriasis, and ankylosing
spondylitis. It prevents the progressive destruction of the
joints in patients with rheumatoid arthritis and the arthritis
of psoriasis.
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Interferon - are multiple
substances naturally produced by cells in the body to help fight
infections and tumors. They may also be synthetic (man-made)
versions of these substances. Alpha interferons, such as Roferon-A,
Intron-A, and Alferon-N, are used to treat hairy cell leukemia,
malignant melanoma, and AIDS-related Kaposi's sarcoma. They are
also used to treat laryngeal papillomatosis (growths in the
respiratory tract) in children, genital warts, and some kinds of
hepatitis. Gamma interferon, like Actimmune, is a synthetic
(man-made) version of a substance naturally produced by cells in
the body to help fight infections and tumors. Gamma interferon
is used to treat chronic granulomatous disease and osteopetrosis.
Interferon beta-1a, like Avonex and Rebif, is used to treat the
relapsing forms of multiple sclerosis (MS) and genital warts.
This medicine will not cure MS, but it may slow some disabling
effects and decrease the number of relapses of the disease.
Interferon beta-1b, such as Betaseron, is also used to treat the
relapsing-remitting form of multiple sclerosis (MS). Again, this
medicine will not cure MS, but may decrease the number of
relapses of the disease. There are no generic forms of
Interferon available.
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Kenalog (Triamcinolone)
- a topical steroid. It reduces or inhibits the actions of
chemicals in the body that cause inflammation, redness, and
swelling. It is used to treat the inflammation caused by a
number of conditions such as allergic reactions, eczema,
and psoriasis.
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Methotrexate - Methotrexate is
classified as an antimetabolite drug, which means it is capable
of blocking the metabolism of cells. It has been found very
helpful in treating
rheumatoid arthritis.
It seems to
work, in part by altering aspects of immune function which may
play a role in causing rheumatoid arthritis.
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Prednisone -
is an oral, synthetic (man-made) corticosteroid used for
suppressing the immune system and inflammation. It has effects
similar to other corticosteroids such as
triamcinolone
(Kenacort),
methylprednisolone
(Medrol), prednisolone (Prelone)
and dexamethasone (Decadron).
These synthetic corticosteroids mimic the action of cortisol
(hydrocortisone), the naturally-occurring corticosteroid
produced in the body by the adrenal glands. Corticosteroids have
many effects on the body, but they most often are used for their
potent anti-inflammatory effects, particularly in those
conditions in which the immune system plays an important role.
Such conditions include arthritis,
colitis, asthma,
bronchitis, certain skin rashes, and allergic or inflammatory
conditions of the nose and eyes. Prednisone is inactive in the
body and, in order to be effective, first must be converted to
prednisolone by enzymes in the liver. Therefore, prednisone may
not work as effectively in people with liver disease whose
ability to convert prednisone to prednisolone is impaired.
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Remicade
(Infliximab) - Infliximab is an injectable antibody
that blocks the effects of tumor necrosis factor alpha (TNF-alpha).
By blocking the action of TNF-alpha, infliximab reduces the
signs and symptoms of inflammation. This medication is
administered via an IV-infusion.
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Sulfasalazine - a prodrug, that is, it is not active
in its ingested form. It is broken down by bacteria in the colon
into two products: 5-aminosalicylic acid (5ASA), and
sulfapyridine. There is some controversy as to which of these
two products are responsible for the activity of azulfidine.
Whereas it is known that 5ASA has therapeutic benefit, it is not
clear whether sulfapyridine adds any further benefit. In the
colon, the products created by the breakdown of sulfasalazine
work as anti-inflammatory agents for treating inflammation of
the colon. The beneficial effect of sulfasalazine is believed to
be due to a local effect on the bowel, although there may also
be a beneficial systemic immune-suppressant effect as well.
Following oral administration, 33% of the sulfasalazine is
absorbed, all of the sulfapyridine is absorbed, and about 33% of
the 5ASA is absorbed. Sulfasalazine was approved by the FDA in
1950.
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CellCept -
contains the active ingredient mycophenolate mofetil. Cellcept
belongs to a group of medicines called immunosuppressants.
Immunosuppressants are used to prevent rejection of transplanted
organs, and work by stopping your immune system from reacting to
the transplanted organ. Cellcept may be used together with other
medicines known as cyclosporin and corticosteroids.
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Thalidomide
- Thalidomide is an oral medication used
for treating the skin conditions of leprosy, a disease caused by
a parasite, Mycobacterium leprae. The mechanism of action of
thalidomide is not well understood. The immune system reaction
to Mycobacterium leprae plays an important role in producing the
skin manifestations of leprosy. Scientists believe that
thalidomide modifies the reaction of the immune system to
Mycobacterium leprae and thereby suppresses the skin reaction.
Thalidomide also is being evaluated as a treatment for HIV.
Thalidomide was approved by the FDA in July, 1998.
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Trental (Pentoxifylline) -
decreases the "stickiness" (viscosity) of blood and thereby
improves its flow. This increase blood flow helps patients with
peripheral arterial disease to obtain better circulation and
oxygen delivery to vital tissues. Pentoxifylline is used in
patients to treat a condition of painful legs that develop with
exercise because of inadequate circulation to the legs and feet.
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Combination
Treatment - Cyclosporine is sometimes used together with
azathioprine when one medication fails to work by itself. A
common combination is prednisone along with an immunosuppressive
drug.
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.
Re-published with kind
permission from the
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