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Antiphospholipid Syndrome (APS): Causes, Tests & Treatment

  • Published: 2009-04-20 (Rev. 2015-05-10) - Contact: Disabled World at Disabled World
  • Synopsis: Antiphospholipid Syndrome is an immune system disorder characterized by excessive blood clotting that may present complications with pregnancy.

Definition: Antiphospholipid Antibody Syndrome

Antiphospholipid Antibody Syndrome is defined as an autoimmune disorder in which the body recognizes certain normal components of blood and/or cell membranes as foreign substances and produces antibodies against them. Antibodies are proteins in the blood and body fluids that bind to foreign invaders like bacteria and viruses and help the immune system destroy and remove them. Sometimes the immune system doesn't function properly and makes antibodies against normal organs and tissues in the body or, in the case of APS, proteins in the blood. There are two known forms of APS. APS may occur in people with systemic lupus or other autoimmune disease, or in otherwise healthy individuals.

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"One of the ways that a person's immune system fights off infections is by producing antibodies, which are proteins in their blood and other body fluids that bind to viruses and bacteria."

Antiphospholipid Syndrome (APS)

Antiphospholipid Syndrome is an immune system disorder characterized by excessive blood clotting that may present complications with pregnancy such as premature birth, miscarriage, or even unexplained fetal death, as well as the presence of antiphospholipid antibodies in the person's blood.

The antiphospholipid antibodies involved include lupus anticoagulant antibodies or cadiolipin. Persons with this syndrome may develop symptoms while these antibodies are detectable in their blood.

One of the ways that a person's immune system fights off infections is by producing antibodies, which are proteins in their blood and other body fluids that bind to viruses and bacteria. These antibodies help the immune system to destroy and remove viruses and bacteria. There are times when the immune system does not function the way it should, and produces antibodies that work against the person's organs and other tissues in their body; these antibodies are referred to as,' auto-antibodies.' The auto-antibodies in APS were at first thought to recognize certain phospholipids, therefore the name, 'antiphospholipid antibodies.' Science now knows that the majority of these auto-antibodies in persons with APS recognize particular blood proteins which bind to phospholipids, not the actual phospholipids themselves.

Antiphospholipid syndrome is also referred to as, 'Phospholipid Antibody Syndrome,' or, 'Hughes Syndrome,' after the doctor who described it. People who do not have this disorder may also have antiphospholipid antibodies in their blood; about two-percent of the population does. Some of these antibodies are harmless, and can be found in association with a number of conditions such as parasitic, bacterial, and viral infections. Certain medications can cause these antibodies to be produced in a person's blood, such as quinine, antibiotics, procainamide, and hydralizine. The illegal drug cocaine can cause these antibodies to be produced in a person's blood as well.

The antiphospholipid antibody is a protein, although it is not considered to be a normal one, and it has been found in people with a variety of illnesses. The illnesses that these people have experienced include stroke, phlebitis, thrombosis, low blood platelet counts, migraine headaches, as well as a rare form of inflammation of the nervous tissues of the spinal cord and brain referred to as, 'Transverse Myelitis.' These antibodies have been found in greater than half of persons with Lupus Erythematosus. Persons with atypical Multiple Sclerosis, as well as those with slowly progressive memory issues also have these antibodies in their blood stream.

Features of APS

People with APS have an increased risk for several different things including issues related to blood clotting. Blood clots in the arteries, clots that go to the person's lungs, or clots in the person's veins are risks. Persons with APS may experience miscarriages that can occur at any stage of pregnancy, although they are more common during the late first trimester or early second trimester. Pre-eclampsia, eclampsia, premature delivery, and fetal growth retardation are complications that may be experienced. Heart attacks, angina, and strokes, as well as transient ischemic attacks and loss of vision are additional risks. Persons with APS may develop heart problems which may require either valve surgery or replacement. They may experience decreased levels of blood platelets that are involved in blood clotting, and they may develop a bluish rash that may be either transient or persistent. The feet or legs of persons with APS may develop skin ulcers.

There are some other features that persons with APS may develop, including neurological symptoms that are similar to Multiple Sclerosis. The person may experience migraine headaches, visual disturbances, and difficulty thinking clearly. Neurological symptoms including dizziness, vision loss, seizures, loss of balance, and abnormal movements may occur as well.

Testing for Antiphospholipid Syndrome

There are a number of different laboratory tests that can detect Antiphospholipid Syndrome. One of these tests is actually a Syphilis test, which returns as false in persons with this syndrome. Another is a Lupus Anticoagulant test; there is also a Prolonged PPT test, and a Cardiolipin test. The presence of anticardiolipin antibodies may indicate Systemic Lupus Erythemotosus; something that is characterized by production of a number of abnormal antibodies.

Causes of Antiphospholipid Syndrome

While the causes of this syndrome are not currently known, what is understood is that the antibodies associated with the syndrome reduce levels of, 'Annexin V;' a protein which bind phospholipids as well as presenting clot-blocking activity within a person. Reduction of Annexin V is believed to be a potential mechanism for an increase in the tendency for a person's blood to clot, as well as the issues related to pregnancies that are characteristic of Antiphospholipid syndrome. Something else that is understood is that anticardiolipin, as well as other antiphospholipid antibodies, are associated with a decrease in levels of prostacyclin. Prostacyclin is a chemical which prevents blood clotting elements from clumping together.

Treating Antiphospholipid Syndrome

Every person with this disorder is treated individually. There are a number of illnesses that are associated with the abnormal grouping of blood clotting elements related to this syndrome, and treatment many times involves prevention of clotting through blood thinning. People with this syndrome tend to form blood clots that can affect the function of nearly every organ in their body. Administration of medications that thin their blood such as Warfarin or Heparin are commonly used. Prednisone and other cortisone medications may also be used in order to suppress the person's immune activity, as well as inflammation, if they present certain features related to the syndrome. Common Aspirin can help by inhibiting the grouping of blood platelets when used in low doses in some people. Persons with Systemic Lupus Erythematosus who have Antiphospholipid syndrome as well may be prescribed Hydroxychloroquinine as added protection against blood clotting.

Additional treatments may involve the use of intravenous gamma globulin for persons with a history of premature miscarriage, or for people who have low amounts of blood platelets during a pregnancy. Some studies have shown that intravenous gamma globulin might not be any more effective than a combination of common aspirin and heparin treatment.

Catastrophic Antiphospholipid Syndrome

Catastrophic Antiphospholipid Syndrome is a type of antiphospholipid syndrome which is characterized by blockages of a variety of blood vessels throughout a persons body. The person's organs may be affected; organs such as the person's heart, kidneys, lungs, bowels, brain, and skin. This type of antiphospholipid syndrome is rare, and is treated with corticosteroids, anticoagulation, as well as plasmapherisis. Catastrophic Antiphospholipid syndrome affects less than one-percent of persons with antiphospholipid syndrome, and is also referred to as, 'Asherson's Syndrome.'

Awareness: Antiphospholipid Syndrome

Burgundy awareness ribbonBurgundy is the awareness ribbon color for Antiphospholipid Syndrome. APS is also referred to as APLS or APLA in the United States and Hughes Syndrome or Sticky Blood in the UK. In the U.S. the month of June is Antiphospholipd Antibody Awareness Month. In the U.K. the month of May is Hughes Syndrome Awareness Month

Other Awareness ribbon colors

Other awareness dates

Statistics: APS

  • 1-5% of the population is believed to have APS.
  • 75-90% of those affected by APS are women.
  • 10-25% of women with recurrent miscarriages have APS.
  • 15-20% of all cases of blood clots in large veins, including blood clots that go to the lungs, are due to APS.
  • One third of strokes occurring in people under the age of 50 are due to APS.
  • 40-50% of persons with lupus also have APS.









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