Antiphospholipid Syndrome – Symptoms, Causes, Prevention and Treatment

Overview and Statistical Facts

Antiphospholipid syndrome (APS), also known as antiphospholipid antibody syndrome or Hughes syndrome, is an autoimmune condition. It is characterized by elevated levels of different antibodies in the blood that results in the formation of clots in blood vessels.

Excessive blood clots in the leg vein can cause deep vein thrombosis. These blood clots can cause damage to the organs depending on its location and the size of the clot. For instance, a clot in the brain can result in a stroke. Pregnant women with APS carry a high chance of having miscarriage, stillbirth and other complications in their pregnancy due to formation of excessive blood clots.

There is no permanent cure for antiphospholipid syndrome, but it can be effectively managed by medications.

Antiphospholipid antibodies are found in about 15-20% of patients suffering from deep vein thrombosis and in one-third of the patients, who develop stroke under the age of 50 years.

According to the APS Foundation of America, about 1-5 % of the general population has APS. It is one of the leading causes of miscarriages and complications in pregnancy when any other cause cannot be identified.

Women are more likely to be affected by this syndrome than men. About 75-90% of the APS patients are women. It is not a rare disease. It is estimated that around 2-6 million women have APS globally. Symptoms first appear in the age group of 20 to 50 years, but in some cases, it can appear in childhood as well.

Types and Symptoms of APS

Types of Antiphospholipid Syndrome:

APS can be of following types:

• Primary APS
• Secondary APS
• Catastrophic APS

Primary APS occurs when there is no other underlying condition responsible for APS.

Secondary APS occurs along with some autoimmune disorders, such as systemic lupus erythematosus (SLE).

Catastrophic APS is a rare case of APS in which there is a rapid organ failure due to generalized thrombosis. It is associated with high risk of death. This condition is also known as Asherson’s syndrome.

Symptoms of Antiphospholipid Syndrome:

General Symptoms of APS include:

• Blood clots in legs
• Repeated miscarriages or stillbirth
• Pain, redness, warmth and swelling in the limbs
• Upper body discomfort in the arms, back, neck and jaw
• Stroke occurring in patients with no risk of developing cardiovascular disease
• Transitory ischemic attack, which lasts for a very short duration without causing any permanent damage to the heart
• Rash with a net-like pattern and is lacy (livedo reticularis)
• Neurological symptoms like chronic headaches, seizures and dementia when the clot hinders blood flow to the brain
• Cardiovascular disease as it can damage heart valves
• Nausea
• Bleeding as the patient develops deficiency of platelets. In such a condition, when the platelet count becomes too low, the person might bleed from his nose and gums. The patient might bleed into his skin, which appears as patches of small red spots, known as petechiae.
• Complications in pregnancy like repeated miscarriages, preeclampsia or pre-term delivery and fetal growth retardation

In extremely rare cases, the person might develop:

• Chorea, in which there is involuntary jerking of body and limbs
• Memory problems
• Mental health problems such as depression, psychosis
• Hearing impairment
• Heart valve problems

Risk Factors of APS

Following are the risk factors associated with APS:

• Genetics
• Lupus, Sjogren’s syndrome or other autoimmune disorders
• Hepatitis C, Syphilis, Parvovirus B19, Cytomegalovirus and other such infections
• Medicines such as hydralazine, phenytoin and quinidine

It is possible to have antibodies associated with APS without developing any symptoms. These antibodies increase the risk of developing blood clots in case of

• Pregnancy
• Immobility due to bed rest, long flight, etc.
• Surgery
• Smoking
• Use of contraceptives or estrogen therapy for menopause
• Increased levels of cholesterol and triglyceride in blood
• Hypertension
• Obesity

Do I have APS?

If you have any autoimmune disorder, then you are at a high risk of developing APS. In such a case, you should get a proper diagnosis for the disorder.

If you observe balance and mobility problems or blood clots in arms and legs, vision problems like double vision, speech and memory problems, increased bruising, minor red spots after long hours of sitting like in a long flight journey or repeated headaches/migraines, then you should visit a medical health practitioner and get your symptoms checked.

If you are a woman and experiencing recurrent early miscarriages usually during the first ten weeks of pregnancy, one or more later miscarriages generally after 10 weeks of pregnancy or a premature birth due to preeclampsia, then you might have high chances of having APS.

Incidentally, these symptoms might also be due to several other conditions, such as thrombocytopenia and deep vein thrombosis. Hence, consulting a medical practitioner is very important for proper diagnosis.

Causes and Prevention of APS

Phospholipids are present in the cell membranes that help in the process of blood clotting. People with APS develop antibodies that attack either the phospholipids or blood proteins that bind to these phospholipids during the process of blood clotting.

When a person suffers from any autoimmune disorder, the immune system mistakenly produces antiphospholipid antibodies. This increases the risk of blood clot formation in the blood vessels; thereby, hindering blood flow to the organs. These antibodies decrease the levels of annexin-V – a cellular protein with clot-blocking properties.

Diagnosis and Tests for APS

1) Medical History

For the diagnosis of APS, a patient must have APS antibody along with a history of health problems associated with the disorder like stroke, heart attack, deep vein thrombosis, pulmonary embolism etc.

It is also known to cause complications in pregnancy.

2) Blood Tests

Blood tests are performed to check the presence of APS antibodies like anticardiolipin, beta-2-glycoprotein I and lupus anticoagulant.

If the test result is positive, a second test is done to confirm APS as the first test result might be due to a short-term infection. Second test is done after 12 or more weeks.

Treatment and Care for APS

1) Standard Initial Treatments

If the patient has thrombosis, a combination of anticoagulants is used:

• Heparin
• Warfarin
• Aspirin

2) Treatment During Pregnancy

Treatment includes:

• Heparin forms, such as enoxaparin and dalteparin
• Aspirin (low dose)

In pregnant women with a history of premature miscarriage, gamma globulins are prescribed. APS patients are at an increased risk of thrombocytopenia – a condition characterized by low level of blood platelets. Steroids are prescribed for treating thrombocytopenia. Immunoglobulins are prescribed to block immune system. For patients with systemic lupus erythematosus, who have APS, hydroxychloroquine is prescribed to protect against blood clotting.

Patients of APS should make it a point to avoid getting bruises or getting on to long flights. They should also take their medicines properly and visit their health provider regularly. Pregnant women should take extra care and get examined more frequently. They should be educated about the symptoms of thrombosis, severe preeclampsia, decreased fetal movement and thromboembolism.

OTC Medications and Self-Management Methods for APS

Aspirin in low doses is available as an over-the-counter drug for managing APS. However, it is still advised to be taken under medical supervision only as it can increase a patient’s chance of profuse bleeding even on minor injury.

For self-management of APS, following steps should be taken:

• Exercising regularly can help the patient in maintaining general health and fitness. It also helps in keeping the heart healthy.
• Consuming a healthy diet

Increased consumption of omega-3 fatty acids in diet can help in reducing the risk for blood clot formation. However, fish oil is known to be rich in vitamin A, which can be harmful for fetal development. Hence, it should be avoided during pregnancy. APS patients should intake a healthy and balanced diet to maintain their general health and lower their risk of developing blood clots.

• Cessation of smoking
• Maintaining healthy weight
• Avoiding alcoholism
• Avoiding long hours of sitting, especially during long flights
• Keeping other conditions such as diabetes and high blood pressure under control
• Taking hormone replacement therapy (HRT) after menopause only under medical supervision
• Taking contraceptive pills after consulting with a medical practitioner

Self-Management During Pregnancy

Pregnant women must consult their doctor about the risks associated with their pregnancy and how to manage it. With proper care, APS patients can carry a full-term pregnancy more successfully than women, whose APS is not managed.

Natural Ways to Cure APS

Following are the natural therapies that can be used to manage APS. They should only be taken after consulting a medical practitioner

Policosanol: It is obtained from the waxy coating of sugarcane and helps in preventing platelet aggregation. However, it should be avoided during pregnancy and breastfeeding.

Garlic: Garlic is known to have anticoagulant properties and can be used for managing APS. Studies have indicated that aged garlic inhibits blood platelet adherence and aggregation. It acts as an anti-inflammatory agent and relaxes the muscles surrounding the blood vessels. This allows the blood to flow more freely and hence, prevent the chances of blood clot formation.

Curcumin: Curcumin, found in turmeric, is known to have blood thinning properties and prevents platelets from clumping together.

Onion: Sulfur compounds present in onion are known to have potential anti-platelet aggregation activity; thus, help in preventing formation of blood clots.

Vitamin E: Consuming foods rich in vitamin E, such as avocados, carrots, spinach, peanuts, olives and walnuts can help reduce the risk of venous thromboembolism.

Health Tip by Experts

It is possible to lead a healthy and normal life with APS. Proper treatment should be followed for managing APS. For secondary APS, the primary disease should be treated. Taking precautions to avoid blood clot formation can help in leading a normal life. Pregnant women with APS must undergo regular health check-ups to ensure proper fetal development.