Antiphospholipid Syndrome

February 3, 2017

Antiphospholipid syndrome (APS), also known as Hughes syndrome, is a systemic autoimmune condition characterized by the presence of antiphospholipid antibodies (APL) in the serum of patients with thrombotic events and/or recurrent pregnancy complications. It is a disorder characterized by recurrent arterial or venous thrombosis and/or pregnancy losses, in the presence of persistently elevated levels of anticardiolipin antibodies and/or evidence of circulating lupus anticoagulant (these abnormalities are detected by blood tests).

Antiphospholipid syndrome is a condition in which the blood is more likely to clot than usual. Because of this, the condition is sometimes called ‘sticky blood’. The clotting can affect any vein, artery or organ in the body and the consequences can include potentially fatal conditions such as heart attacks, strokes, and DVT. In pregnancy, the antibodies can cause miscarriage, pre-eclampsia, small babies, early deliveries and stillbirth.

As blood flows through the entire body, Hughes syndrome can present a wide variety of symptoms, not all of them as serious as stroke or heart attack.

People from all ages, including children can have Hughes syndrome, but it tends to mainly affect the 20-50 year old age group and women more so than men. In the UK alone, it is estimated that Hughes syndrome affects 1 in 100 people.

Types o APS

  • Primary APS – The individual has no known autoimmune disease present, other than APS. This type accounts for more than 50% of all cases.
  • Secondary APS – The individual has already been diagnosed with systemic lupus erythematosus (SLE) or another underlying immune disorder. More women than men have this type of APS which matches the statistics for SLE and other overlapping connective tissue diseases.

What happens in APS?

All cells in the body have membranes made of phospholipids, a class of lipids (fatty acids), that hold the cell together. Within these phospholipids, antibodies play a role in how quickly or slowly blood clots. In APS, the body produces antiphospholipid antibodies that mistakenly attack the body’s own phospholipids. An antibody is a protein that is produced by the plasma (blood) cells An and used by the immune system. The antibodies seek out and target antigens (foreign objects such as viruses and bacteria). Some examples of antigens include: microorganisms like bacteria, fungi, parasites, viruses, and chemicals.

The purpose of these proteins (antibodies) within phospholipids is to control how quickly or slowly blood clots. As a result, a patient suffering with antiphospholipid syndrome or phospholipid antibody syndrome has an abnormally higher chance of blood clotting or narrowed blood vessels.


Genetic Factors – Research into the genetics around APS is still at an early stage, but it seems the genes a person inherits from his or her parents may play a role in the development of abnormal antiphospholipid antibodies. APS isn’t passed down directly from parents to children in the same way as other conditions, such as haemophilia and sickle cell anaemia. However, having a family member with antiphospholipid antibodies increases the chance of the immune system also producing them.

Antiphospholipid antibodies – Antibodies are proteins produced by the immune system to help fight off infection and illness. They’re part of the body’s defence system and produced to help protect against ‘foreign invaders’, such as bacteria and viruses. Antibodies signal the immune system to release chemicals to kill these bacteria and viruses and to prevent infection spreading. It’s not known how this causes the blood to clot more easily. However, most experts believe that keeping the blood at the correct consistency (not too runny and not too sticky) is a delicate balancing act that relies on different types of proteins and fats working together. This balance may be disrupted by the abnormal antibodies in people with APS.

Environmental factors – It’s thought that one or more environmental triggers may be needed to trigger APS in some people. Environmental factors that may be responsible include –

  • Viral infections, such as the cytomegalovirus (CMV) or parvovirus B19
  • Bacterial infections, such as E. coli (a bacteria often associated with food poisoning) or leptospirosis (an infection usually spread by certain animals)
  • Certain medications, such as anti-epileptic medicine or the oral contraceptive pill

Who is at risk?

People who have APS antibodies but no signs or symptoms are at risk of developing APS. Health problems, other than autoimmune disorders, that can trigger blood clots include –

  • Smoking
  • Prolonged bed rest
  • Pregnancy and the postpartum period
  • Birth control pills and hormone therapy
  • Cancer and kidney disease


Major signs and symptoms of blood clots include –

  • Chest pain and shortness of breath
  • Pain, redness, warmth, and swelling in the limbs
  • Ongoing headaches
  • Speech changes
  • Upper body discomfort in the arms, back, neck, and jaw
  • Nausea


Other complications include –

  • Vascular Thrombosis – It may appear for the first time as a blood clot in an artery or vein (this is called a vascular thrombosis).
  • Embolism – xIt may appear as a blockage of a blood vessel that has been caused by a clot that has traveled in the blood stream from another location in the body ( this is called an embolism).
  • Pregnancy Complications – A woman who has recurring pregnancy loss may find out through testing that APS might be the underlying cause.Antiphospholipid Syndrome
  • Thrombocytopenia or (Immune Thrombocytopenia) – This is a condition associated with APS that means that a person has low platelets. Platelets are little pieces of blood cells that help wounds heal and blood to clot. If a person has a low platelet count, the person can be at risk for mild to serious bleeding.
  • Rashes or skin conditions – Some people may develop a rash that can be described as red with a mottled or lacy, net-like pattern. This is called livedo reticularis. Others may have ulcers or sores, most often on the legs, and others may have necrosis (a condition when the skin tissue dies).
  • Stroke or other neurological disorders – Embolic stroke (caused by a blood clot that travels to the brain) or cerebrovascular thrombosis (a blood clot that forms in the brain) can occur. Please see the signs regarding ‘when to see a doctor’ below. Seizures, migraines, other cognitive dysfunctions can also be indicators of those with APL antibodies, although studies are still being done to prove the link between those conditions and the occurrence of APS strokes.
  • Lupus and other autoimmune disorders – Because APS is classified as an immune disorder (when the immune response attacks the body’s healthy tissue), those with APL or antiphospholipid syndrome sometimes have an additional immune disorder. Although there are other disorders on the list, systemic lupus erythematosis is the most common autoimmune disorder linked to those with APL.


  • Abdominal pain
  • Coma
  • Confusion
  • Edema (swelling) in the extremities (ankles, feet or hands)
  • Fits (seizures)
  • Progressive breathlessness
  • Tiredness


Medication – The treatment plan will be specific to the individual and their current health status with regards to the APS symptoms that they are experiencing. Ideally, any treatment regimen will be directed towards thinning (anticoagulating) the blood to prevent clotting. These include –

  • Warfarin (Coumadin)
  • Heparin
  • Aspirin
  • Prednisone (used to suppress the immune system activity and inflammation)
  • For those with SLE who have APS, hydroxychoroquine or Plaquenil can also add some protection against blood clots.
  • For those who have aPL antibodies but have not shown symptoms, low-dose aspirin is generally recommended by the treating physician.
  • For women who wish to get pregnant, but have had a history of miscarriage, the physician may prescribe heparin (prevents blood clots) and low-dose aspirin.

Prophylactic therapy – Eliminate other risk factors, such as oral contraceptives, smoking, hypertension, or hyperlipidemia. Prophylaxis is needed during surgery or hospitalization, as well as management of any associated autoimmune disease.

Alternative Treatment

Policosanol – Policosanol is a natural product made from the waxy coating of sugar cane that helps lower cholesterol. Various studies have investigated the effect of policosanol on platelet aggregation.

Aortic acid – Aortic extract is usually made from the hearts of animals, usually sheep, cows, or pigs. There are many substances in this extract, including aortic acid, which is a broad term encompassing several constituents. Mesoglycan is the most studied of these constituents. Further research is needed to determine whether or not aortic acid may help treat patients with deep vein thrombosis (DVT).

Garlic – The garlic bulb is made of many garlic cloves that are wrapped in a paper-thin, white skin. Garlic, which is often used as a spice in food, has also been used to treat many medical conditions. Garlic has been studied in humans as a possible anticoagulant therapy.

Pycnogenol – Pycnogenol is the patented trade name for a water extract of the bark of the French maritime pine tree (Pinus pinaster ssp. atlantica), which is grown in coastal southwestern France.

Rutin – Rutin is a yellow crystalline flavonol glycoside that occurs in various plants, especially the buckwheat plant, black tea, apple peels, onions, and citrus. Superficial vein thrombosis (SVT) is a common complication of varicose veins.

Seaweed, kelp, bladderwrack – Bladderwrack (Fucus vesiculosus) is a brown seaweed found along the northern coasts of the Atlantic and Pacific oceans and North and Baltic seas. Another seaweed that grows alongside bladderwrack is Ascophyllum nodosum, andit is often combined with bladderwrack in kelp preparations.

Nutritional Supplements – Certain herbal and vitamin supplements are known to cause serious drug interactions with warfarin. Herbal products and supplements including stomach remedies, multivitamins, green tea products and garlic are most likely to result in dangerous drug interactions.


Reference –