What Is Antiphospholipid Antibody Syndrome?
Antiphospholipid (AN-te-fos-fo-LIP-id) antibody
syndrome (APS) is an autoimmune disorder. Autoimmune disorders occur when the
immune system makes antibodies that attack and damage the bodys tissues
or cells by mistake. Antibodies are a type of protein that the immune system
usually makes to defend against infection.
In APS, the body mistakenly makes antibodies that
attack phospholipidsa type of fat. Phospholipids are found in all living
cells and cell membranes, including blood cells and the lining of blood
vessels.
When antibodies attack phospholipids, they damage
cells. This causes unwanted blood clots to form in the bodys arteries and
veins. (These are the vessels that carry blood to your heart and body.)
Usually, blood clotting is a normal bodily process.
Blood clots help seal small cuts or breaks and prevent you from losing too much
blood. In APS, however, too much blood clotting can block blood flow and damage
the bodys organs.
Overview
Some people have APS antibodies, but dont ever
have signs or symptoms of the disorder. The presence of APS antibodies, by
itself, doesnt mean that you have APS. To be diagnosed with APS, you must
have APS antibodies and a history of health problems related to the
disorder.
APS can lead to a number of health problems, such as
stroke,
heart
attack, kidney damage,
deep
vein thrombosis (throm-BO-sis),
pulmonary
embolism (PULL-mun-ary EM-bo-lizm), or pregnancy-related problems.
Pregnancy-related problems may include multiple
miscarriages, a miscarriage late in pregnancy, or a premature birth due to
eclampsia. (Eclampsia, which follows
preeclampsia, is a serious condition that causes seizures in
pregnant women.)
Very rarely, some people who have APS develop many
blood clots within weeks or months. This condition is called catastrophic
antiphospholipid syndrome (CAPS).
People who have APS also are at higher risk for
thrombocytopenia
(THROM-bo-si-to-PE-ne-ah). This is a condition in which your blood has a low
number of blood cells called platelets (PLATE-lets). This can lead to mild to
serious bleeding.
In APS, thrombocytopenia occurs because the
platelets are used up by the clotting process or because antibodies destroy
them.
In some cases, APS can be fatal. This may occur due
to large blood clots or blood clots in the heart, lungs, or brain.
Outlook
APS can affect people of any age. However, its
more common in women and people who have other autoimmune or rheumatic
disorders, such as
lupus. ("Rheumatic" refers to disorders that affect the
joints, bones, or muscles.)
APS has no cure, but medicines can help prevent its
complications. Medicines are used to stop blood clots from forming and keep
existing clots from getting larger. Treatment for the disorder is long
term.
If you have APS and another autoimmune disorder,
its important to control that condition as well. When the other condition
is controlled, APS may cause fewer problems.
Other Names for Antiphospholipid Antibody
Syndrome
- Antiphospholipid syndrome
- aPL syndrome
- Anticardiolipin antibody syndrome, or aCL
syndrome
- Lupus anticoagulant syndrome
- Hughes syndrome
- Sneddon syndrome
What Causes Antiphospholipid Antibody
Syndrome?
Antiphospholipid antibody syndrome (APS) occurs when
the body's immune system makes antibodies (proteins) that attack
phospholipids.
Phospholipids are a type of fat found in all living
cells and cell membranes, including blood cells and the lining of blood
vessels. What causes the immune system to make antibodies against phospholipids
isn't known.
APS causes unwanted blood clots to form in the
body's arteries and veins. Usually, blood clotting is a normal bodily process.
It helps seal small cuts or breaks and prevents you from losing too much blood.
In APS, however, too much blood clotting can block blood flow and damage the
bodys organs.
Researchers don't know why APS occurs. Some believe
that the antibodies damage or affect the inner lining of the blood vessels,
causing blood clots to form. Others believe that the immune system makes
antibodies in response to blood clots damaging the blood vessels.
Who Is At Risk for Antiphospholipid Antibody
Syndrome?
Antiphospholipid antibody syndrome (APS) can affect
people of any age. The disorder is more common in women than men, but it
affects both sexes.
APS also is more common in people who have other
autoimmune or rheumatic disorders, such as
lupus. About 10 percent of all people who have lupus also have
APS. About half of all people who have APS also have another autoimmune or
rheumatic disorder.
What Are the Signs and Symptoms of Antiphospholipid
Antibody Syndrome?
The signs and symptoms of antiphospholipid antibody
syndrome (APS) are related to abnormal blood clotting. The outcome of a blood
clot depends on its size and location.
Blood clots can form in or travel to the arteries or
veins in the brain, heart, kidneys, lungs, and limbs. Clots can limit or block
blood flow. This can damage the bodys organs and may cause death.
Major Signs and Symptoms
Major signs and symptoms of blood clots include:
- Chest pain and shortness of breath
- Pain, redness, warmth, and swelling in the
limbs
- Ongoing headaches
- Speech changes
- Upper body discomfort in the arms, back, neck,
and jaw
- Nausea (feeling sick to your stomach)
Blood clots can lead to
stroke,
heart
attack, kidney damage,
pulmonary
embolism, and
deep
vein thrombosis.
Pregnant women who have APS can have successful
pregnancies. However, they're at higher risk for miscarriages, stillbirths, and
other pregnancy-related problems, such as
preeclampsia (pre-e-KLAMP-se-ah).
Preeclampsia is high blood pressure that occurs
during pregnancy. This condition may progress to eclampsia. Eclampsia is a
serious condition that causes seizures in pregnant women.
Some people who have APS also have
thrombocytopenia.
This is a condition in which your blood has a low number of blood cells called
platelets. Mild to serious bleeding causes the main
signs
and symptoms of thrombocytopenia. Bleeding can occur inside the body
(internal bleeding) or on the skin.
Other Signs and Symptoms
Other symptoms of APS include chronic headaches,
memory loss, or
heart
valve disease. Some people who have the disorder also get a lacy-looking
red rash on their wrists and knees.
How Is Antiphospholipid Antibody Syndrome
Diagnosed?
Your doctor will diagnose antiphospholipid antibody
syndrome (APS) based on your medical history and the results from
blood
tests.
Specialists Involved
People who have APS often are treated by a
hematologist (a blood disease specialist). You may have APS and another
autoimmune disorder, such as
lupus. If so, you also may be treated by a doctor who
specializes in that disorder.
Many autoimmune disorders that occur with APS also
affect the joints, bones, or muscles. Rheumatologists specialize in treating
these types of disorders.
Medical History
Some people have APS antibodies, but dont ever
have signs or symptoms of the disorder. The presence of APS antibodies, by
itself, doesnt mean that you have APS.
To be diagnosed with APS, you must have APS
antibodies and a history of health problems related to the disorder. These
health problems may include
stroke,
heart
attack, kidney damage,
deep
vein thrombosis (throm-BO-sis),
pulmonary
embolism (PULL-mun-ary EM-bo-lizm), or pregnancy-related problems.
Pregnancy-related problems may include multiple
miscarriages, a miscarriage late in pregnancy, or a premature birth due to
eclampsia. (Eclampsia, which follows
preeclampsia, is a serious condition that causes seizures in
pregnant women.)
Blood Tests
Your doctor can use blood tests to confirm a
diagnosis of APS. These tests check your blood for any of the three APS
antibodies: anticardiolipin, B2 glycoprotein I, and lupus anticoagulant.
The term "anticoagulant" refers to a substance that
prevents blood clotting. It may seem odd that one of the APS antibodies is
called lupus anticoagulant. This is because the antibody slows clotting in lab
tests. However, in the human body, it increases the risk for blood clots.
To test for the APS antibodies, a small amount of
blood is taken from your body. It's often drawn from a vein in your arm using a
small needle. The procedure usually is quick and easy, but it may cause some
short-term discomfort, such as a slight bruise.
You may need a second blood test to confirm positive
results. This is because a single positive test can result from a short-term
infection. The second blood test often is done 12 weeks or more after the first
one.
Some healthy people may test positive for APS
antibodies but have no signs or symptoms of the disorder. The presence of the
APS antibodies, by itself, doesn't mean that you have APS.
How Is Antiphospholipid Antibody Syndrome
Treated?
Antiphospholipid antibody syndrome (APS) has no
cure, but some medicines can help prevent complications. The goals of treatment
are to prevent blood clots from forming and keep existing clots from getting
larger.
If you have APS and another autoimmune disorder,
such as
lupus, its important to control that condition as well.
When the other condition is controlled, APS may cause fewer problems.
Research is ongoing for new ways to treat APS.
Medicines
Anticoagulants, or "blood thinners," are used to
stop blood clots from forming. They also keep existing blood clots from getting
larger. These medicines are taken as either a pill, an injection under the
skin, or through a needle or tube inserted into a vein (called intravenous, or
IV, injection).
Warfarin and heparin are two blood thinners used to
treat people who have APS. Warfarin is given in pill form.
(Coumadin® is a common brand name for warfarin.) Heparin is
given as an injection or through an IV tube. There are different types of
heparin. Your doctor will discuss the options with you.
Your doctor may treat you with both heparin and
warfarin at the same time. Heparin acts quickly. Warfarin takes 2 to 3 days
before it starts to work. Once the warfarin starts to work, the heparin is
stopped.
Sometimes aspirin is used with warfarin. In other
cases, aspirin may be used alone. Aspirin also thins the blood and helps
prevent blood clots.
Blood thinners dont prevent or treat APS. They
simply reduce the risk of further blood clotting. Treatment with these
medicines is long term. Discuss all treatment options with your doctor.
Side Effects
The most common side effect of blood thinners is
bleeding. This happens if the medicine thins your blood too much. This side
effect can be life threatening.
Sometimes the bleeding is internal (inside your
body). People treated with blood thinners usually need regular blood tests,
called PT and PTT tests, to check how well their blood is clotting.
These tests also show whether you're taking the
right amount of medicine. Your doctor will check to make sure that youre
taking enough medicine to prevent clots, but not so much that it causes
bleeding.
Talk to your doctor about the warning signs of
internal bleeding and when to seek emergency care. (For more information, see
"Living With Antiphospholipid Antibody
Syndrome.")
Treatment During Pregnancy
Pregnant women who have APS can have successful
pregnancies. With proper treatment, women who have APS are more likely to carry
their babies to term.
Pregnant women who have APS usually are treated with
heparin or heparin and low-dose aspirin.
Babies whose mothers have APS are at higher risk for
slowed growth while in the womb. If youre pregnant and have APS, you may
need to have extra ultrasound tests (sonograms) to check the fetus
growth. This test uses sound waves to look at organs and structures inside your
body.
Treatment for Other Medical Conditions
People who have APS are at increased risk for
thrombocytopenia.
This is a condition in which your blood has a low number of blood cells called
platelets. Platelets help the blood clot. If you have APS, you will need
regular
complete
blood counts (a type of blood test) to count the number of platelets in
your blood.
Thrombocytopenia is treated with medicines and
medical procedures. For more information, see
"How
Is Thrombocytopenia Treated?"
If you have other health problems, such as heart
disease or diabetes, work with your doctor to manage them.
Living With Antiphospholipid Antibody Syndrome
Antiphospholipid antibody syndrome (APS) has no
cure. However, you can take steps to control the disorder and prevent
complications.
Take all medicines as your doctor prescribes, get
ongoing medical care, and talk to your doctor about healthy lifestyle changes
and other concerns.
Medicines
You may need to take anticoagulants, or "blood
thinners," to prevent blood clots or to keep them from getting larger. You
should take these medicines exactly as your doctor prescribes.
Tell your doctor about all other medicines
youre taking, including over-the-counter or herbal medicines. Some
medicines, including over-the-counter ibuprofen or aspirin, can thin your
blood. Taking two medicines that thin your blood may increase your risk for
bleeding.
Ongoing Medical Care
If you have APS, its important to get regular
medical checkups. Have blood tests done as your doctor directs. These tests
help track how well your blood is clotting.
The medicines used to treat APS may cause bleeding.
Bleeding can occur inside your body (internal bleeding) or on its surface. Know
the warning signs of internal bleeding, so you can get help right away. They
include:
- Unexplained bleeding from the gums and nose
- Increased menstrual flow
- Bright red vomit or vomit that looks like coffee
grounds
- Bright red blood in your stools or black, tarry
stools
- Pain in your abdomen or severe pain in your
head
- Sudden changes in vision
- Sudden loss of movement in your limbs
- Memory loss or confusion
A lot of bleeding after a fall or injury or easy
bruising or bleeding also may mean that your blood is too thin. Talk to your
doctor about these warning signs and when to seek emergency care.
Lifestyle Changes
Talk to your doctor about lifestyle changes that can
help you stay healthy. Ask him or her whether your diet may interfere with your
medicines. Some foods or drinks may increase or decrease the effects of
warfarin.
Discuss with your doctor what amount of alcohol is
safe for you to drink if you're taking medicine. If you smoke, ask your doctor
about programs and products that can help you quit. Smoking can damage your
blood vessels and raise your risk for a number of health problems.
APS medicines may increase your risk for bleeding.
Thus, your doctor may advise you to avoid activities that have a high risk of
injury, such as some contact sports.
Other Concerns
Pregnancy
APS can raise your risk for a number of
pregnancy-related problems. Talk to your doctor about how to manage your APS if
your pregnant or planning a pregnancy.
With proper treatment, women who have APS are more
likely to carry babies to term than women whose APS isnt treated.
Birth Control
Women who have APS shouldnt use birth control
or hormone therapy that contains estrogen. Estrogen increases the risk for
blood clots. Talk to your doctor about other birth control methods.
Surgery
If you need surgery, your doctor may adjust the
amount of medicines you take before, during, and after the surgery to prevent
dangerous bleeding.
Key Points
- Antiphospholipid antibody syndrome (APS) is an
autoimmune disorder. Autoimmune disorders occur when the immune system makes
antibodies that attack and damage the bodys tissues or cells by mistake.
- In APS, the body makes antibodies that attack
phospholipidsa type of fat. This can cause blood clots to form in the
bodys arteries and veins. Blood clots can limit or block blood flow and
damage the bodys organs.
- Some people have the APS antibodies, but
dont ever have signs or symptoms of the disorder. The presence of APS
antibodies, by itself, doesnt mean that you have APS. To be diagnosed
with APS, you must have APS antibodies and a history of health problems related
to the disorder.
- APS can lead to a number of health problems, such
as
stroke,
heart
attack, kidney damage,
deep
vein thrombosis, or
pulmonary
embolism. In women, APS raises the risk for miscarriages, stillbirths, and
other pregnancy-related problems. In some cases, APS can cause death.
- APS can affect people of any age. The disorder is
more common in women and people who have other autoimmune or rheumatic
disorders, such as
lupus.
- The signs and symptoms of APS are related to
abnormal blood clotting. The outcome of the blood clot depends on its size and
location.
- Common signs and symptoms of blood clots include
chest pain and shortness of breath; pain, redness, warmth, and swelling in the
limbs; ongoing headaches; speech changes; upper body discomfort in the arms,
back, neck, and jaw; and nausea (feeling sick to your stomach).
- Your doctor will diagnose APS based on your
medical history and the results from
blood
tests. Blood tests can confirm if you have any APS antibodies.
- APS has no cure. However, some medicines can help
prevent complications. Anticoagulants, or "blood thinners," are used to stop
blood clots from forming. These medicines also keep existing clots from getting
larger.
- The most common side effect of blood thinners is
bleeding. This happens if the medicine thins your blood too much. Bleeding can
occur inside your body (internal bleeding) or on the skin. Talk to your doctor
about the warning signs of internal bleeding and when to seek emergency
care.
- If you have APS, its important to take all
of your medicines as your doctor prescribes, get ongoing medical care, and talk
to your doctor about healthy lifestyle changes and other concerns.
- Research is ongoing for new ways to treat
APS.
Links to Other Information About Antiphospholipid
Antibody Syndrome
Non-NHLBI Resources
Clinical Trials
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