Antiphospholipid Syndrome

What is antiphospholipid syndrome (APS)?

Antiphospholipid syndrome (APS) is a condition that makes your body much more likely than usual to form blood clots. Healthcare providers sometimes call it antiphospholipid antibody syndrome.

The blood clots from APS can cause severe, life-threatening complications like strokes. They can also make pregnant people much more likely to experience miscarriages and other pregnancy complications.

APS is an autoimmune disease. Autoimmune diseases happen when your immune system accidentally attacks your body instead of protecting it.

What are antiphospholipid syndrome symptoms?

Some people with antiphospholipid syndrome notice mottled skin (livedo reticulitis) with a darkened, lace-like pattern. But most people with APS don’t experience any noticeable symptoms until they have a blood clot or experience a pregnancy complication.

Symptoms of a blood clot can include:

• Chest pain.
• Shortness of breath (dyspnea).
• Frequent headaches.
• Sudden pain in your arms, legs, back, neck or jaw.
• Redness or swelling in your arm or leg.
• Abdominal pain (a stomachache).

Blood clots can be life-threatening emergencies. Go to the emergency room right away if you think you have a blood clot.

APS symptoms you might not be able to see, feel or notice can include having:

• Low blood platelet levels.
• Anemia.
• Heart valve abnormalities.

What causes antiphospholipid syndrome?

Antiphospholipid syndrome happens when your immune system accidentally starts making certain abnormal antibodies. Usually, your immune system makes and uses antibodies to protect you. They’re special proteins that act like an army of security robots that find and destroy germs, allergens or toxins in your blood. When your immune system detects a new unwanted substance in your body, it makes antibodies customized to find and destroy that invader.

Antiphospholipid syndrome gets its name from how your immune system attacks your body. If you have APS, your immune system makes antibodies that attack proteins bound to phospholipids (a type of fat cell). These attacks damage the phospholipids and make them more likely to clump together and form clots.

The three antibodies that can cause APS include:

• Lupus anticoagulant.
• Anticardiolipin.
• Anti-B2 glycoprotein 1.

People with APS have one, two or all three of these antiphospholipid antibodies. It’s also possible to have antiphospholipid antibodies and never have signs or symptoms of APS. Even though experts know they cause APS, they aren’t sure what triggers your immune system to start making these faulty antibodies.

What are the risk factors?

Anyone can develop APS, but you may be more likely to if you:

• Are assigned female at birth (AFAB).
• Are between 30 and 40 years old.
• Have other autoimmune diseases like lupus or rheumatoid arthritis.
• Have close family members (including biological parents and grandparents) with APS.

What are APS syndrome complications?

Antiphospholipid syndrome can cause severe, potentially fatal complications.

The most severe APS complications happen when blood clots block blood vessels throughout your body, including in your:

• Brain (strokes).
• Heart (thrombosis).
• Lungs (pulmonary embolism).
• Kidneys.

Pregnant people with APS can experience a miscarriage if a clot blocks the flow of nutrients in the placenta. It can also increase your risk of preeclampsia.

Catastrophic antiphospholipid syndrome (CAPS) is an APS complication that causes multiple blood clots to form in different organs throughout your body within a few days of each other. CAPS is fatal in almost half of people who experience it. Fewer than 1% of people with APS develop CAPS.

What is the life expectancy of someone with antiphospholipid syndrome?

APS doesn’t usually affect how long you’ll live (your life expectancy). It can cause fatal complications if you experience a blood clot that blocks a blood vessel in one of your organs, especially if you have CAPS.

How do healthcare providers diagnose antiphospholipid syndrome?

Most people are only tested for APS after a blood clot or miscarriage. Some people have antiphospholipid antibodies but never experience APS symptoms or complications.

A healthcare provider will diagnose APS with blood tests.

Your provider will take samples of your blood for three different blood tests — one test that screens for each of the three antiphospholipid antibodies. At least one of the blood tests must come back positive for APS two different times three months or more apart for you to be diagnosed with antiphospholipid syndrome.

What is the best treatment for antiphospholipid syndrome?

Your provider will suggest treatments that lower your risk of having a blood clot. The most common treatments include anticoagulant medications (blood thinners), including:

• Heparin: This is an intravenous (IV) medication that helps break up clots while you have one. A provider will usually give it to you if you’ve been hospitalized for having a blood clot.
• Warfarin: This is a medication you’ll take orally (by mouth) that thins your blood and helps prevent clots before they form.
• Aspirin: Your provider may suggest you take over-the-counter (OTC) or prescription aspirin to prevent clots in your arteries.

Your provider might recommend medications to prevent miscarriages if you’re pregnant and have APS, including:

• Enoxaparin: This is a medication your provider will inject under your skin. They might suggest you take a low-dose aspirin, too.
• Intravenous immunoglobulin (IVIG): IVIG can reduce your risk of getting sick and lessen the severity of illnesses when you do get them.
• Corticosteroids: Corticosteroids are prescription medications that reduce inflammation and calm (suppress) your immune system.

These treatments are safe for pregnant people and shouldn’t harm you or the fetus.

Blood thinner side effects

Taking blood thinners increases your chances of bleeding (both internally and outside your body).

Contact your healthcare provider right away if you experience any of the following symptoms:

• Unexplained bleeding from your gums or nose.
• A heavier menstrual period.
• Vomit that’s bright red or looks dark like coffee grounds.
• Bright red blood in your poop or having black, tarry poop.
• Pain in your abdomen or severe head pain.
• Sudden changes in your vision.
• Suddenly being unable to move your arms or legs.

Can I prevent antiphospholipid syndrome?

Because experts aren’t sure what makes your body produce the antiphospholipid antibodies that cause it, there’s no way to prevent APS.

What can I expect if I have APS?

APS responds well to treatment with blood thinners, and you should be able to do all your usual activities once you start treatment. Most people managing APS with blood thinners can safely get pregnant and have biological children.

Does antiphospholipid syndrome go away?

APS doesn’t usually go away. You may never have a clot or experience complications, but you’ll probably need to take blood thinners for the rest of your life.

How do I take care of myself if I have antiphospholipid syndrome?

The most important part of living with APS is preventing blood clots. Taking blood-thinning medication is a big part of that prevention.

Managing other health conditions that can increase your clot risk is also important. Talk to your provider about your unique clot risks if you have:

• Diabetes.
• High blood pressure (hypertension).
• High cholesterol.
• Obesity.
• Other autoimmune diseases.

Smoking also increases your risk of blood clots, especially if you have a clotting disorder like APS.

People who take estrogen therapy (for birth control or during menopause) also have an increased clot risk.

What foods should you avoid if you have APS?

People with APS don’t usually need to follow a special eating plan. The same kinds of food that help you maintain good overall health should help support your body if you have APS.

You may need to monitor how much vitamin K you eat if you take warfarin. Suddenly eating a lot of foods high in vitamin K (like leafy greens) when you usually don’t can increase your clot risk. Talk to your provider about any foods you should avoid.

Ask your provider how much alcohol is safe for you to drink. Alcohol thins your blood, and this effect can be increased when you’re taking blood thinners.

When should I see my healthcare provider?

You’ll need to see your provider regularly to make sure your blood is still able to clot safely if you get a cut or bruise.

Antiphospholipid syndrome can increase the risk of pregnancy-related complications. Talk to your healthcare provider if you’re concerned about any risks or pregnancy complications you might experience.

When should I go to the emergency room?

Go to the ER right away if you:

• Think you have a blood clot.
• Experience any symptoms of a clot.
• Are bleeding and can’t control it.

Which questions should I ask my provider?

It may be helpful to ask your provider:

• Do I have APS or another clotting disorder?
• Which medications will help me prevent blood clots?
• How often will I need follow-up appointments?
• What are my risks for pregnancy complications?
• Which warning signs of complications or clots should I watch out for?

Does having antiphospholipid syndrome (APS) mean I have lupus?

Although part of the testing for APS is called “lupus anticoagulant,” your healthcare provider isn’t specifically testing for lupus when ordering tests for APS. Some people with lupus also have APS, which is where this term originated.