What is antiphospholipid syndrome (APS)?
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 (such as anti-cardiolipin or lupus anticoagulant antibodies) in the blood.
Clotting disorders associated with antiphospholipid syndrome include stroke, blood clots deep within the legs (deep venous thrombosis, or DVT) and clots in the lungs (pulmonary embolism, or PE). Patients with antiphospholipid syndrome have both blood clots and antiphospholipid antibodies that are detectable with blood testing.
Antiphospholipid syndrome is also called phospholipid antibody syndrome, cardiolipin antibody syndrome, and Hughes syndrome in honor of the doctor who first described it.
What causes antiphospholipid syndrome?
The cause of antiphospholipid syndrome is not completely known. Antiphospholipid antibodies reduce the levels of annexin V, a protein that binds phospholipids and has potent clot-blocking (anticoagulant) activity. The reduction of annexin V levels is thought to be a possible mechanism underlying the increased tendency of blood to clot and the propensity to pregnancy loss characteristic of the antiphospholipid syndrome. Certain genes may be risk factors for the development of antiphospholipid syndrome.
Antiphospholipid antibodies, such as anticardiolipin, have also been associated with decreased levels of prostacyclin, a chemical that prevents the clumping together of normal blood clotting elements called platelets.
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 a small percentage 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 and HIV), and parasitic (malaria) infections. Certain drugs can cause antiphospholipid antibodies to be produced in the blood, including antibiotics, cocaine, hydralazine, procainamide, and quinine.
Nevertheless, the antiphospholipid antibodies (a protein) are not considered normal blood proteins and have been found to be associated with a number of illnesses. These illnesses include abnormal clotting (thrombosis) of arteries (stroke and infarction) and/or veins, 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.
Researchers are recently also finding that there are patients with slowly progressive memory problems and patients with a form of "atypical multiple sclerosis" who have antiphospholipid antibodies detectable in their blood.
Diagnosis of antiphospholipid syndrome
Patients with the antiphospholipid syndrome can have a variety of antibodies to molecules called phospholipids in their blood. These antibodies include VDRL/RPR (a syphilis test that can be falsely positive in these patients), lupus anticoagulant, prolonged PTT, beta 2 glycoprotein I antibodies, and anticardiolipin antibody. As mentioned above, the anticardiolipin antibody has also been found in patients with the immune disease systemic lupus erythematosus, which is characterized by the production of a variety of abnormal antibodies.
What is the treatment for antiphospholipid syndrome?
The treatment of patients with anticardiolipin syndrome has substantially evolved since cardiolipin antibodies were noted to be clinically important in the mid-1980s. Each manifestation of the antiphospholipid syndrome, and each individual patient with the condition, is treated uniquely.
Because many of the features of illness with anticardiolipin syndrome are associated with an abnormal grouping of normal blood clotting elements (platelets), treatment is often directed toward preventing clotting by thinning the blood. Patients with this disorder have a tendency to form blood clots (thrombosis). The unwanted blood clotting can affect the function of virtually any organ. Medications that thin (anticoagulate) the blood, such as heparin (Hep-Lock, Liquaemin, Lovenox) and warfarin (Coumadin), are powerful blood thinners that are used for treatment. Aspirin has an effect on platelets that inhibits their grouping (aggregation) and has also been used in low doses to thin the blood of selected patients with less severe disease. Cortisone-related medications, such as prednisone, have been used to suppress the immune activity and inflammation in patients with certain features of the condition. For patients with systemic lupus erythematosus who also have antiphospholipid syndrome, hydroxychloroquine (Plaquenil) has been reported to add some protection against blood clotting.
Other reported treatments include the use of intravenous gamma globulin for selected patients with histories of premature miscarriage and those with low blood-clotting elements (platelets) during pregnancy. Recent research studies, however, suggest that intravenous gamma globulin may be no more effective than a combination of aspirin and heparin.
The treatment of antiphospholipid antibody syndrome during pregnancy typically involves low dose aspirin and low molecular weight heparin (Lovenox). Additionally, intravenous immunoglobulins have been infused, but their effectiveness is not proven.

QUESTION
The term arthritis refers to stiffness in the joints. See AnswerComplications and catastrophic antiphospholipid syndrome
Catastrophic antiphospholipid syndrome (CAPS) is a variant of antiphospholipid syndrome that is characterized by blockage of many blood vessels throughout the body. As a result of catastrophic antiphospholipid syndrome, many organs can be affected, including the skin, lungs, brain, heart, kidneys, and bowels. Catastrophic antiphospholipid syndrome is treated with anticoagulation, corticosteroids (cortisone medication), and plasmapheresis (plasma exchange).
Catastrophic antiphospholipid syndrome is rare. Catastrophic antiphospholipid syndrome is sometimes referred to as Asherson's syndrome after the researcher who described it in the early 1990s.
What is the prognosis for antiphospholipid syndrome?
The prognosis of antiphospholipid syndrome depends on the character and the intensity of its manifestations. Earlier treatment will tend to have better outcomes.
Is it possible to prevent antiphospholipid syndrome?
If a person is already known to have phospholipid antibodies, it is possible to prevent antiphospholipid syndrome with methods that decrease the chances of blood clotting, including aspirin and/or heparin. Hydroxychloroquine (Plaquenil) may have some preventative benefits for selected patients.
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Bucciarelli S., et al. "Mortality in the Catastrophic Antiphospholipid Syndrome." Arthritis & Rheumatism (2006): 2568-2576.
Pisetsky, David S., and Peter H Schur. "Diagnosis of the antiphospholipid syndrome." UpToDate.com. Sept. 17, 2014. <http://www.uptodate.com/contents/diagnosis-of-the-antiphospholipid-syndrome>.
Ruddy, Shaun, eds., et al. Kelley's Textbook of Rheumatology. Philadelphia: WB Saunders Co., 2000.
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aspirin
Aspirin (Aspirin, Arthritis Foundation Safety Coated Aspirin, Bayer Aspirin, Bayer Children's Aspirin, Ecotrin, and many others) is a NSAID used to treat fever, pain, and inflammation in the body that results from forms of arthritis, and soft tissue injuries. Aspirin is also used for decreasing the risk of heart attacks and strokes. Side effects, drug interactions, pregnancy information, and pregnancy safety information should be reviewed prior to taking any medication.Complete Blood Count (CBC)
A complete blood count (CBC) is a calculation of the cellular makeup of blood. A CBC measures the concentration of white blood cells, red blood cells, platelets in the blood, and aids in diagnosing conditions and disease such as malignancy, anemia, or blood clotting problems.Deep Vein Thrombosis (DVT, Blood Clot in the Legs)
Deep vein thrombosis (DVT) is a blood clot in the deep veins, and can be caused by broken bones, trauma to a limb, immobility, medications, smoking, cancer, genetic predisposition, and cancer. Symptoms and signs of a deep vein thrombosis in a leg are swelling, tenderness, redness, warmth, and pain. Treatments for DVT include medications and surgery.Human Immunodeficiency Virus (HIV)
HIV (human immunodeficiency virus) infection left untreated causes AIDS (acquired immunodeficiency syndrome). The human immunodeficiency virus (HIV) is a type of virus called a retrovirus, which can infect humans when it comes in contact with tissues that line the vagina, anal area, mouth, or eyes, or through a break in the skin. HIV infection is generally a slowly progressive disease in which the virus is present throughout the body at all stages of the disease. Three stages of HIV infection have been described. The initial stage of infection (primary infection), which occurs within weeks of acquiring the virus, often is characterized by the flu- or mono-like illness that generally resolves within weeks. The stage of chronic asymptomatic infection (meaning a long duration of infection without symptoms) lasts an average of eight to 10 years without treatment. The stage of symptomatic infection, in which the body's immune (or defense) system has been suppressed and complications have developed, is called the acquired immunodeficiency syndrome (AIDS). The symptoms are caused by the complications of AIDS, which include one or more unusual infections or cancers, severe loss of weight, and intellectual deterioration (called dementia). When HIV grows (that is, by reproducing itself), it acquires the ability to change (mutate) its own structure. These mutations enable the virus to become resistant to previously effective drug therapy. The goals of drug therapy are to prevent damage to the immune system by the HIV virus and to halt or delay the progress of the infection to symptomatic disease. Therapy for HIV includes combinations of drugs that decrease the growth of the virus to such an extent that the treatment prevents or markedly delays the development of viral resistance to the drugs. The best combination of drugs for HIV are those that effectively suppress viral replication in the blood and also are well tolerated and simple to take so that people can take the medications consistently without missing doses.Malaria
Malaria is a disease that is spread by the bite of an infected Anopheles mosquito. Malaria symptoms include fever, chills, nausea, vomiting, and body aches. Treatment involves supportive care and antibiotics.Migraine Headache
Migraine headache is a type of headache associated with a sensitivity to light, smells, or sounds, eye pain, severe pounding on one side of the head, and sometimes nausea and vomiting. The exact cause of migraine headaches is not known. Triggers for migraine headaches include certain foods, stress, hormonal changes, strong stimuli (loud noises), and oversleeping. Treatment guidelines for migraines include medicine, pain management, diet changes, avoiding foods that trigger migraines, staying hydrated, getting adequate sleep, and exercising regularly. Prevention of migraine triggers include getting regular exercise, drinking water daily, reducing stress, and avoiding trigger foods.Miscarriage
Miscarriage is the medical term for the spontaneous loss of pregnancy from conception to 20 weeks gestation. Risk factors for a woman having a miscarriage include cigarette smoking, older maternal age, radiation exposure, previous miscarriage, maternal weight, illicit drug use, use of NSAIDs, and trauma or anatomical abnormalities to the uterus. There are five classified types of miscarriage: 1) threatened abortion; 2) incomplete abortion; 3) complete abortion; 4) missed abortion; and (5 septic abortion. While there are no specific treatments to stop a miscarriage, a woman's doctor may advise avoiding certain activities, bed rest, etc. If a woman believes she has had a miscarriage, she needs to seek prompt medical attention.
Pregnancy Planning (Tips)
Pregnancy planning is an important step in preparation for starting or expanding a family. Planning for a pregnancy includes taking prenatal vitamins, eating healthy for you and your baby, disease prevention (for both parents and baby) to prevent birth defects and infections, avoiding certain medications that may be harmful to your baby, how much weight gain is healthy exercise safety and pregnancy, travel during pregnancy.Rheumatology and Rheumatologist
Rheumatology is the study of rheumatic diseases and conditions. Rheumatologists are internal medicine physicians who treat these illnesses, in particular arthritis.Stroke
A stroke is an interruption of the blood supply to part of the brain caused by either a blood clot (ischemic) or bleeding (hemorrhagic). Symptoms of a stroke may include weakness, numbness, double vision or vision loss, confusion, vertigo, difficulty speaking, or understanding speech. A physical exam, imaging tests, neurological exam, and blood tests may be used to diagnose a stroke. Treatment may include administration of clot-busting drugs, supportive care, and in some instances, neurosurgery. The risk of stroke can be reduced by controlling high blood pressure, high cholesterol, diabetes, and stopping smoking.Lupus (Systemic Lupus Erythematosus or SLE)
Systemic lupus erythematosus is a condition characterized by chronic inflammation of body tissues caused by autoimmune disease. Lupus can cause disease of the skin, heart, lungs, kidneys, joints, and nervous system. When only the skin is involved, the condition is called discoid lupus. When internal organs are involved, the condition is called systemic lupus erythematosus (SLE).Lupus Pictures 1
Systemic Lupus Erythematosus. Systemic lupus erythematosus is the most common form of the autoimmune disease lupus. This red scaly rash on a person's cheek is typical. SLE affects the skin and other internal organs. It’s possible for the heart, lungs and brain to become involved, followed by the formation of scar tissue (fibrosis). SLE is usually treated with corticosteroids.Ultrasound
Ultrasound (and ultrasonography) is imaging of the body used in the medical diagnosis and screening of diseases and conditions such as:- TIAs,
- stroke,
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- heart valve irregularities,
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- heart disease,
- gallstones,
- kidney stones,
- liver disease,
- diseases of the female reproductive, and
- diseases of the male reproductive organs.
Hepatitis (Viral Hepatitis A, B, C, D, E, G)
Hepatitis is most often viral, due to infection with one of the hepatitis viruses (A, B, C, D, E, F (not confirmed), and G) or another virus (such as those that cause infectious mononucleosis, cytomegalovirus disease). The main nonviral causes of hepatitis are alcohol and drugs. Many patients infected with hepatitis A, B, and C have few or no symptoms of illness. For those who do develop symptoms of viral hepatitis, the most common are flu-like symptoms including: loss of appetite, nausea, vomiting, fever, weakness, tiredness, and aching in the abdomen. Treatment of viral hepatitis is dependent on the type of hepatitis.
warfarin (Coumadin, Jantoven)
Warfarin (Coumadin, Jantoven) is an anticoagulant drug that inhibits the blood from clotting, thus preventing blood clots. It is prescribed for the treatment of patients with deep vein thrombosis, the reduction of pulmonary embolism, and in patients with atrial fibrillation to reduce the risk of strokes and heart attack. Common side effects of warfarin include:
- Rash
- Hair loss
- Diarrhea
- Jaundice
- Bloating
Drug interactions, and warnings and precautions, and pregnancy and breastfeeding information should be reviewed prior to taking this medication.