Antiphospholipid Syndrome

Medical Author: William C. Shiel Jr., MD, FACP, FACR
Medical Editor: Kenneth Kaye, MD

• What is antiphospholipid syndrome?
• What laboratory tests can support the diagnosis of antiphospholipid syndrome?
• What causes antiphospholipid syndrome?
• How is antiphospholipid syndrome treated?
• What is catastrophic antiphospholipid syndrome?
• Antiphospholipid Syndrome At A Glance

What is antiphospholipid syndrome?

The antiphospholipid syndrome is a disorder of the immune system that is characterized by excessive clotting of blood and/or certain complications of pregnancy (premature miscarriages, unexplained fetal death, or premature birth) and the presence of antiphospholipid antibodies (cardiolipin or lupus anticoagulant antibodies) in the blood. Patients with antiphospholipid syndrome have developed abnormal symptoms while having antiphospholipid antibodies that are detectable with blood testing.

Antiphospholipid syndrome is also called phospholipid antibody syndrome. Antiphospholipid syndrome has been referred to as Hughes syndrome in honor of the doctor who first described it.

It is important to note that antiphospholipid antibodies can also be found in the blood of individuals without any disease process. In fact, antiphospholipid antibodies have been reported in approximately 2% of the normal population. Harmless antiphospholipid antibodies can be detected in the blood for a brief period occasionally in association with a wide variety of conditions, including bacterial, viral (hepatitis, HIV), and parasite (malaria) infections. Certain drugs can cause antiphospholipid antibodies to be produced in the blood, including antibiotics, cocaine, hydralazine, procainamide, and quinine.

Nevertheless, the antiphospholipid antibody (a protein) is not considered a normal blood protein and has been found in patients to be associated with a number of illnesses. These illnesses include abnormal clotting (thrombosis) of arteries (stroke, infarction) and/or veins (phlebitis), premature miscarriages (spontaneous abortions), abnormally low blood platelet counts (thrombocytopenia), purplish mottling discoloration of the skin (livedo reticularis), migraine headaches, and a rare form of inflammation of the nervous tissue of the brain or spinal cord, called transverse myelitis. Antiphospholipid antibodies have also been detected in over half of patients with the immune disease systemic lupus erythematosus.

Researchers are recently also finding that there are patients with slowly progressive memory problems and patients with a form of "atypical multiple sclerosis" and antiphospholipid antibodies detectable in their blood.

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