Antiphospholipid syndrome (APS) is an autoimmune disease in which blood clots form in the veins and arteries. These clots, or thromboses, can develop in the brain, lungs, legs, and other organs, which can lead to conditions such as heart attack or stroke. Although there’s no cure, medications such as blood thinners can help prevent clots from forming.
What Is Antiphospholipid Syndrome?
Antiphospholipid syndrome is a disease in which the body’s immune system mistakenly produces antibodies that attack certain parts of cells called phospholipids.
When these phospholipids become damaged, blood clots can develop in the veins or arteries, leading to a stroke or heart attack. It can also lead to miscarriages, stillbirth, or other pregnancy complications, such as preeclampsia.
Antiphospholipid syndrome, which is also sometimes known as antiphospholipid antibody syndrome, “sticky blood syndrome,” or Hughes syndrome, is a leading cause of stroke in younger people under age 50.
Some people with high levels of these antibodies never develop symptoms. In other cases, some signs of antiphospholipid syndrome may not be detected until a person experiences problems such as a miscarriage or heart attack.
The signs and symptoms that may appear include:
Blood clots (especially in the legs)
Low blood platelet levels
Anemia
Purplish skin discoloration that looks blotchy or lacey (livedo reticularis)
Skin ulcers
Repeated miscarriages, stillbirths, or premature delivery
Preeclampsia during pregnancy
Heart disease
Stroke
The symptoms of a blood clot include:
Chest pain
Shortness of breath
Nausea
Pain, redness, or swelling in the arm or leg
Frequent headaches
Discomfort in the arms, back, neck, or jaw
Abdominal pain
It’s not known exactly what causes antiphospholipid syndrome, although certain gene variants have been linked to the condition. These variants may then prompt certain people to produce abnormal antibodies — specifically, the three antibodies lupus anticoagulant, anticardiolpin, and anti-B2 glycoprotein 1 — that then cause antiphospholipid syndrome.
APS is thought to be caused by a combination of genetic factors and environmental exposure, such as to a virus. In most cases, APS impacts people who have no family history of the disease, though there have been rare cases of APS occurring in multiple members of a family.
Antiphospholipid syndrome is more common in women than men. People with autoimmune and rheumatic diseases, such as lupus, are also more likely to have the condition.
If your doctor suspects you may have antiphospholipid syndrome, you may be given blood tests to check for the antibodies associated with the condition. To be given a diagnosis, you must test positive for the antibodies more than once, in tests given at least 12 weeks apart.
Even if you test positive for the antibodies, however, you may not have antiphospholipid disorder, particularly if you’re not experiencing related conditions such as blood clots or frequent miscarriages.
Antiphospholipid syndrome is typically treated with medications called blood thinners (anticoagulants and antiplatelets), which can help slow down or prevent blood clots from forming. Common medications used to treat APS include warfarin (Coumadin) and aspirin.
In the case of an acute blood clot (which needs immediate medical attention because the clot can potentially travel to the lungs, blocking blood flow), an injection of heparin (an anticoagulant) may be given in the hospital.
Since untreated antiphospholipid syndrome can increase the risk of miscarriage during pregnancy, pregnant people may be given low-dose aspirin as well as injections of enoxaparin (an anticoagulant) to prevent fetal loss. In more serious cases of recurrent miscarriages, doctors may recommend corticosteroids such as prednisone or intravenous immunoglobulin infusions.
In some cases, such as when blood thinners are not effective in treating APS, doctors may turn to targeted immunosuppressants such as rituximab and eculizumab to treat APS, though more research is needed to determine the effectiveness of these medications in managing APS.
Some other drugs that are used to treat APS include hydroxychloroquine (a malaria medication that's long been used to treat rheumatic diseases) and statins (cholesterol-lowering medications that may have anti-inflammatory and anticoagulant effects in people with APS).
There’s no cure for antiphospholipid syndrome, and it’s not possible to prevent the disorder. But by taking medication and making certain lifestyle changes, you can reduce the risk of blood clots.
Engaging in regular exercise, eating a healthy diet, and practicing other health-promoting habits can reduce your risk of high blood pressure and diabetes, which can increase your risk for stroke.
If you’re taking blood thinners to prevent blood clots, you may need to make certain changes to your lifestyle to reduce the risk of bleeding. These may include avoiding sports or activities that could cause bruising or injury, and using caution around sharp tools, such as knives or scissors.
Certain supplements and medications can also interfere with anticoagulants such as warfarin. You may also need to avoid or cut back on alcohol, which can increase the effect of blood-thinning medications.
Foods that are high in vitamin K (which is important for blood clotting) can interfere with medications such as warfarin, so you'll want to avoid eating large amounts of vitamin K-rich foods such as:
Avocado
Broccoli
Brussels sprouts
Cabbage
Leafy greens
Garbanzo beans
Some supplements and medications that may interfere with warfarin include:
Certain pain relievers
Certain cold medicines
Some stomach remedies
Turmeric
Multivitamins
Garlic
Ginkgo
Green tea
Your doctor may also advise you to take steps that would lower your risk of blood clots, which may include:
Maintaining a healthy A1C level, if you have diabetes
With treatment and healthy lifestyle habits, people with antiphospholipid syndrome can expect to live generally healthy lives. Some studies have shown that antiphospholipid syndrome is associated with up to a 94 percent survival rate after 10 years.
The condition can cause permanent health problems, however: Research has found that more than 30 percent of people with the condition experience organ damage and more than 20 percent have serious complications that include pulmonary hypertension (high blood pressure in the lungs) and impaired kidney function after 10 years.
Antiphospholipid syndrome can cause blood clots, which can lead to stroke and other heart problems, as well as pregnancy complications such as miscarriage and stillbirth.
In rare cases (fewer than 1 percent), people with the condition can also experience a complication called catastrophic antiphospholipid syndrome (CAPS), which can be fatal in more than half of all cases. This occurs when blood clots develop in multiple blood vessels in the body, blocking blood flow and causing organ failure, typically in the kidneys, lungs, brain, heart, and liver.
Antiphospholipid syndrome is believed to affect about 1 in every 2,000 people in the United States. The disorder impacts more women than men: As many as 75 to 90 percent of those affected by APS are women.
One out of five women who have a history of recurrent miscarriages have antiphospholipid syndrome.
About 40 percent of people with lupus (systemic lupus erythematosus) also have antiphospholipid syndrome.
Antiphospholipid syndrome (also known as APS) is an autoimmune disease in which the immune system mistakenly attacks certain parts of cells in the body, which can eventually lead to blood clots.
The condition can lead to stroke or heart attack, as well as pregnancy complications that include preeclampsia and miscarriage.
Although there’s no cure, antiphospholipid syndrome can often be managed successfully by taking blood thinner medication.
