Antiphospholipid syndrome is a condition that can cause clotting within your arteries or veins and various other problems, some life-threatening.
Antiphospholipid syndrome may cause clots to form in your legs, a condition known as deep vein thrombosis (DVT). Antiphospholipid syndrome may also cause blood clots to form in organs, such as your kidneys or lungs. Damage depends on the extent and location of the clot. For instance, a clot in your brain can cause stroke.
In antiphospholipid syndrome, your immune system mistakenly produces antibodies to certain proteins in your blood. Antibodies are specialized proteins that normally attack body invaders, such as viruses and bacteria.
There's no cure for antiphospholipid syndrome, but medications can be effective in reducing your risk of blood clots.
The signs and symptoms you develop with antiphospholipid syndrome depend on where clots form or travel to. A clot that forms or a traveling clot (embolus) may cause:
~Blood clots in your legs (deep vein thrombosis, or DVT)
~Blood clots that travel into your lungs (pulmonary embolism)
~Repeated miscarriages or stillbirths and other complications of pregnancy, such as premature delivery and high blood pressure during pregnancy (preeclampsia)
Other less common signs and symptoms include:
~Neurological symptoms. Chronic headaches, including migraines, dementia and seizures are possible when a blood clot obstructs blood flow to parts of your brain.
~Rash. Some people develop a red rash with a lacy, net-like pattern (livedo reticularis) on their wrists and knees.
~Cardiovascular disease. About one in three people with antiphospholipid syndrome have heart valve abnormalities. Heart valves open and close to keep blood flowing through your heart's four chambers in only one direction. Typically, the mitral valve — the valve between your heart's upper left and lower left chambers — develops masses or thickens, which can cause blood to leak backward through it (regurgitation). The aortic valve — the valve between your heart's lower left chamber and aorta — also may be affected.
~Bleeding. Some people experience a decrease in platelets, blood cells necessary for normal clotting. If you have this condition (thrombocytopenia), you may have few or no symptoms. However, if your platelet count drops too low, you may have episodes of bleeding, particularly from your nose and gums. You can also bleed into your skin, which will appear as patches of small, red spots (petechiae).
Infrequent signs and symptoms include:
~Movement disorder, in which your body and limbs jerk uncontrollably (chorea)
~Cognitive problems, such as poor memory
~Sudden hearing loss
~Mental health problems, such as depression or psychosis
When to see a doctor
If you already have an autoimmune condition, talk to your doctor about whether you should be tested for antiphospholipid antibodies. Other reasons to contact your doctor include:
~Pain or swelling in your leg or arm. See your doctor especially if your vein is red, swollen or tender. Seek emergency care if vein swelling and pain are severe or are accompanied by a high fever or shortness of breath, which could indicate DVT and an increased chance of a blood clot traveling to your lungs (pulmonary embolism).
■Vaginal spotting or bleeding during the first 20 weeks of your pregnancy. This may be a sign of miscarriage; however, many women spot or bleed without miscarrying. If you've had repeated pregnancy losses or unexplained severe complications of pregnancy, it could be related to antiphospholipid syndrome. Talk to your doctor about whether testing would be right for you.
If you have antiphospholipid syndrome and you're thinking of attempting pregnancy, treatments are available during your pregnancy. But be sure to seek the care of an expert obstetrical provider to discuss your options.
When it's an emergency
Seek emergency care if you have certain other serious signs and symptoms. Look for:
■Signs and symptoms of stroke. These include sudden numbness, weakness or paralysis of your face, arm or leg; sudden difficulty speaking or understanding speech; sudden visual disturbances; sudden, severe headache, and dizziness.
■Signs and symptoms of pulmonary embolism. These include sudden shortness of breath, chest pain and coughing up blood-streaked sputum.Causes
The role of phospholipids
When you bleed, such as from a cut or during menstruation, your body protects you from losing too much blood by the process of clotting (coagulation). Clotting is a complex process, involving a number of steps and several bodily substances.
Phospholipids, which make up cell membranes, are one of the substances involved. If you have antiphospholipid syndrome, your antibodies mistakenly attack either your phospholipids or proteins in your blood that bind to your phospholipids. Classifications
There are two main classifications of antiphospholipid syndrome:
■Primary. If you have no other autoimmune disorder, such as systemic lupus erythematosus (SLE), you have primary antiphospholipid syndrome.
■Secondary. If you do have lupus or another autoimmune disorder, your antiphospholipid syndrome is secondary.
With secondary antiphospholipid syndrome, the cause is considered to be your lupus or other autoimmune disorder.
The cause of primary antiphospholipid syndrome is unknown. However, some factors are associated with developing antiphospholipid antibodies — though not necessarily developing the syndrome. They include:
■Infections. People with syphilis, HIV infection, hepatitis C and malaria, among others, have a higher incidence of having the antibodies.
■Medications. Taking certain drugs, such as the high blood pressure medication hydralazine, the anti-seizure medication phenytoin (Dilantin) and the antibiotic amoxicillin (Amoxil, Trimox), may lead to an increased risk.
■Genetic predispositions. Although the disorder isn't considered hereditary, research indicates that relatives of people with antiphospholipid syndrome are more likely to have the antibodies.
Risk factors for antiphospholipid syndrome include:
■Having an autoimmune condition, such as lupus or Sjogren's syndrome.
This increases your risk of developing antiphospholipid antibodies.
■Having certain infections, such as syphilis or hepatitis C.
■Taking certain medications, such as hydralazine for high blood pressure.
■Having a family member with antiphospholipid syndrome.
Antiphospholipid syndrome occurs most frequently in young to middle-aged women, although it can occur at any age and also affects men. Risk factors for developing symptoms
It's possible to have the antibodies associated with antiphospholipid syndrome without ever developing signs or symptoms. However, if you have these antibodies, your risk of developing blood clots increases particularly if you:
■Remain immobile for a period of time (such as sitting during a long airline flight)
■Have high blood pressure or high cholesterol
■Take oral contraceptives
Depending on which organ is affected by a blood clot and how severe the obstruction of blood flow to that organ is, untreated antiphospholipid syndrome can lead to permanent damage or death. Complications may include:
■Kidney failure. This can result from decreased blood flow to your kidneys.
■Stroke. Decreased blood flow to a part of your brain can cause a stroke, which can result in permanent neurological damage, such as partial paralysis and loss of speech (aphasia).
■Cardiovascular problems. If a clot forms in your leg (deep vein thrombosis), the clot can damage the valves in the veins in your affected leg, which normally serve to keep the blood flowing upward to your heart. This may result in a condition called chronic venous insufficiency, which causes chronic swelling and discoloration in your lower legs, because of the impaired blood flow upward to your heart. Another possible complication is heart damage.
■Lung problems. Complications related to your lungs may include high blood pressure in your lungs (pulmonary hypertension) and pulmonary embolism.
■Pregnancy complications. These may include miscarriages, stillbirths, premature delivery and high blood pressure during pregnancy (preeclampsia).
Preparing for your appointment
In most cases, it's the complications of antiphospholipid syndrome — such as deep vein thrombosis, stroke or pregnancy loss — that will direct you to seek medical care. Depending on your complication, you'll likely be meeting with a specialist. For deep vein thrombosis, for instance, you'll meet with a hematologist. For stroke, you'll see a neurologist and for pregnancy loss or complications, you'll meet with your obstetrician or gynecologist.
If you have time, prepare for your appointment by making a list of any symptoms you've noticed. It's also a good idea to write down your questions.
Your doctor will have questions, too. To help diagnose antiphospholipid syndrome, your doctor may ask:
■Do you have a history of stroke or blood clots?
■Do you have a history of pregnancy complications, such as high blood pressure, miscarriage or stillbirth?
■Do you have lupus or another autoimmune disorder?
■Have you experienced frequent headaches or migraines?
■Have you noticed a red, net-like rash on your wrists or knees?
Tests and diagnosis
If you have one or more episodes of thrombosis or pregnancy loss, your doctor can confirm that you have antiphospholipid syndrome with a blood test that detects the presence of the antibodies. To confirm a diagnosis of antiphospholipid syndrome, the antibodies must appear in your blood at least twice, in tests conducted 12 weeks apart. Treatments and drugs
Doctors generally use medications that reduce your blood's tendency to clot, such as low-dose aspirin, to treat antiphospholipid syndrome.
If you have thrombosis, standard initial treatment initially involves a combination of heparin and warfarin. Your doctor will discontinue the heparin after the initial treatment and continue the warfarin, possibly for the rest of your life. Your doctor may also recommend adding low-dose aspirin. Treatment during pregnancy
Anticoagulation therapy is particularly complex during pregnancy. The therapy is expensive, requires regular injections and carries some significant risks of side effects.
~Heparin. This anticoagulant medication works more quickly than warfarin, but, until recently, it had to be administered through a vein and monitored closely. Some forms of heparin — enoxaparin (Lovenox) and dalteparin (Fragmin) — are known as low molecular weight heparin, which you can inject yourself. Heparin is considered safe to take during pregnancy.
~Warfarin (Coumadin). This anticoagulant comes in pill form, so it's easier to take than heparin is. Rarely, warfarin can cause birth defects, so it isn't usually recommended during pregnancy. Rarely, a doctor may prescribe warfarin during pregnancy, but only if the benefits of using it outweigh the risks.
Though anticoagulation therapy during pregnancy may be complicated, the good news is that it usually prevents antiphospholipid syndrome-related miscarriages.
If you're taking anticoagulant medication, your doctor will monitor your dosage with blood tests to be sure your blood is capable of clotting enough to stop your bleeding if you bruise or cut yourself.
Lifestyle and home remedies
If your antiphospholipid syndrome requires that you take anticoagulant medication, take extra precautions to keep from injuring yourself and to avoid bleeding. Follow these suggestions:
~Avoid playing contact sports or engaging in other activities that could be dangerous or could cause you to fall.
~Use a softer toothbrush and waxed floss.
~Shave with an electric razor.
~Take extra care when using knives, scissors and other sharp tools.
If you have antiphospholipid antibodies but do not take anticoagulant medication, take these precautions:
~Tell any doctors performing your medical care that you have antiphospholipid antibodies.
~Ask your health care providers to take measures to help prevent deep vein thrombosis if you are immobilized due to surgery or other medical reasons.
~Take steps, such as reducing your cholesterol, to help prevent heart attack and stroke.
April 4, 2009
© 1998-2009 Mayo Foundation for Medical Education and Research (MFMER). All rights reserved.
Kathy A. Walters
Founder of LupusMCTDLupusWebsite@aol.com
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