What Is Antiphospholipid Syndrome?

Antiphospholipid syndrome is an autoimmune disorder that causes an increased tendency to form blood clots. Blood clots, also known as thrombi, are the body’s way to control bleeding. They are gel-like clumps of blood cells that block a blood vessel and stop bleeding. 

People with antiphospholipid syndrome have an abnormal protein in the blood that causes too many blood clots to form. This can lead to serious complications like heart attack and stroke. 

Antiphospholipid syndrome usually affects people assigned female at birth between the ages of 30 and 40. The first sign of this disorder is often a pregnancy complication caused by blood clots. It affects about 1 in 2,000 people in the United States. About 70% of them were assigned female at birth. 

A hematologist (a medical doctor who specializes in treating blood disorders) and a rheumatologist (a medical doctor who specializes in treating autoimmune conditions) usually work together to manage antiphospholipid syndrome with medications, such as blood thinners, and lifestyle recommendations. 

Symptoms

Antiphospholipid syndrome causes blood clots that lead to symptoms. Some people with this syndrome do not develop any symptoms of blood clots. However, it is important to be aware of the signs so that you can seek treatment right away if you think you have it. 

A blood clot (thrombus) in the arm or leg may look like an area of redness that is swollen and warm to the touch. It is usually painful as well. 

Symptoms of a serious complication caused by a blood clot may include:

• Migraine headaches
• Changes in speech 
• Chest pain
• Shortness of breath 
• Nausea 
• Open sores
• Red rash on the wrists or knees
• Memory loss 

What Causes Antiphospholipid Syndrome? 

Antiphospholipid syndrome occurs when your immune system produces high amounts of antiphospholipid antibodies. Antibodies are proteins in the blood that detect and destroy foreign particles like bacteria and viruses. In people with autoimmune syndromes, the antibodies attack healthy cells and tissue. People who have high levels of antiphospholipid antibodies are at an increased risk of blood clots.

Researchers aren’t certain what causes this immune reaction but believe it could be triggered in response to an infection. The body normally makes antiphospholipid antibodies in response to foreign germs (pathogens). If the pathogens appear similar to the body’s own cells, the antibodies may attack healthy cells, leading to blood clotting. This may happen after an infection or during pregnancy.

Risk Factors

While researchers are not certain of the exact cause of antiphospholipid syndrome, there are known risk factors. Risk factors include:

• Autoimmune syndrome: It is estimated that up to 40% of people with lupus also test positive for high levels of antiphospholipid antibodies.
• Pregnancy: People who have been pregnant are at an increased risk due to changes in the immune system caused by pregnancy.
• Family history: Antiphospholipid syndrome can run in families, but most cases are considered random or sporadic. 
• Certain infections: The immune reactions to human immunodeficiency virus (HIV), hepatitis C, and Lyme disease may increase your risk of producing too many antiphospholipid antibodies. 
  

Diagnosis

A primary care provider is usually the first one to notice the signs of antiphospholipid syndrome. They will likely order blood tests if you develop signs of a blood clot. Possible signs include redness and swelling in an extremity, new-onset headaches, and chest pain. Antiphospholipid syndrome also causes pregnancy complications such as repeated miscarriages. 

If your healthcare provider suspects a problem with blood clotting, they will order the following tests:

• Complete blood count (CBC): This is to check the levels of platelets and red blood cells.
• Antiphospholipid antibodies: The presence of anticardiolipin, beta-2 glycoprotein I, and lupus anticoagulant could indicate antiphospholipid syndrome. 
  

Treatments for Antiphospholipid Syndrome  

Once your primary care provider determines that you are at risk of antiphospholipid syndrome, they will likely refer you to both a hematologist and a rheumatologist. There is no cure for antiphospholipid syndrome, and the goal of treatment is to manage the syndrome and prevent complications. Your healthcare team will work with you to develop a treatment plan to prevent new blood clots and keep any current ones from growing. 

Your provider will likely recommend taking a blood thinning medication every day. Options include:

• Coumadin (warfarin)
• Plavix (clopidogrel)
• Heparin
• Aspirin

If you are pregnant, your healthcare team will develop a plan to lower your risk of blood clots that could lead to miscarriage. Your treatment plan will likely include:

• Blood thinners: Low-dose aspirin to prevent blood clots 
• Corticosteroids: Steroids to develop the baby’s lungs in case of preterm birth
• Immunoglobulin: Antibody therapy to manage your immune response 
  

How to Prevent Blood Clots  

Antiphospholipid syndrome is an autoimmune condition that cannot be prevented. Fortunately, there are steps that you can take to lower your risk of blood clots, including:

• Maintaining a healthy weight
• Engaging in physical activity every day
• Eating a nutritious diet
• Avoiding smoking 
• Talking with your healthcare provider about a safe contraception plan (because hormonal birth control may raise your risk of clots)
  

Related Conditions 

Antiphospholipid syndrome causes blood clots that can lead to serious complications. The most common sites for clots are the legs and brain but clots can occur anywhere in the body. Complications of antiphospholipid syndrome may include:

• Heart attack: This can be caused by a blood clot in the heart.
• Stroke: This is characterized by a blood clot in the brain.
• Pulmonary embolism: This is characterized by a blood clot in the lung.
• Pulmonary hypertension: This is caused by high blood pressure in your lungs.
• Mesenteric thrombosis: This is caused by a blood clot in the major vessel that drains your intestines.
• Hepatic veno-occlusive disease: This is characterized by a blood clot in the liver.

People who have untreated antiphospholipid syndrome while they are pregnant are at higher risk of experiencing the following pregnancy complications:

• Preeclampsia: Blood pressure condition that occurs during pregnancy
• Underdeveloped placenta: When the placenta (the organ that grows in the uterus during pregnancy to provide nutrients and oxygen to the baby) does not receive enough blood flow 
• Preterm birth: Early delivery of a baby
• Miscarriage: Pregnancy loss  
  

Living With Antiphospholipid Syndrome

There is no cure for antiphospholipid syndrome, but it can be managed with treatment. The 10-year survival rate for this syndrome is 90% to 94%. Antiphospholipid syndrome can significantly impact your quality of life. It is estimated that more than 30% of people with this syndrome will develop permanent organ damage, and 20% will develop a severe disability in the 10 years after diagnosis. 

To manage antiphospholipid syndrome, focus on strategies to prevent blood clots from forming. Work with your healthcare team on a treatment plan that includes the use of long-term blood thinners. Aim to maintain a healthy weight, eating a nutrient-dense diet, exercise regularly, and avoid tobacco smoke.