- Antiphospholipid Syndrome Center
- Rheumatoid Arthritis Slideshow Pictures
- Joint-Friendly Exercises to Reduce RA Pain Slideshow
- Take the RA Quiz
- Patient Comments: Antiphospholipid Syndrome - Symptoms
- Patient Comments: Antiphospholipid Syndrome - Cause
- Patient Comments: Antiphospholipid Syndrome - Effective Treatments
- Patient Comments: Antiphospholipid Syndrome - Tests
- Find a local Rheumatologist in your town
- Antiphospholipid syndrome facts
- What is antiphospholipid syndrome? What are antiphospholipid syndrome symptoms and signs?
- What are the causes and risk factors of antiphospholipid syndrome?
- What laboratory tests can support the diagnosis of antiphospholipid syndrome?
- How is antiphospholipid syndrome treated?
- What is the treatment for antiphospholipid syndrome during pregnancy?
- What is catastrophic antiphospholipid syndrome?
- What is the prognosis of antiphospholipid syndrome?
- Is it possible to prevent antiphospholipid syndrome?
Antiphospholipid syndrome facts
- Antiphospholipid syndrome is an autoimmune disorder that can affect virtually any organ.
- Patients with antiphospholipid syndrome can have a variety of antibodies to phospholipids in their blood.
- Antiphospholipid syndrome involves excessive blood clotting.
- Each individual patient with the antiphospholipid syndrome is treated uniquely according to what symptoms are present. The mainstay of therapy is anticoagulation.
What is antiphospholipid syndrome? What are antiphospholipid syndrome symptoms and signs?
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 (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.
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. 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" who have antiphospholipid antibodies detectable in their blood.
Quick GuideLupus Symptoms, Rash, and Treatment
What are the causes and risk factors of 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.
What laboratory tests can support the 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.
How is antiphospholipid syndrome treated?
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.
What is the treatment for antiphospholipid syndrome during pregnancy?
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.
What is 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 of 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.
John A. Daller, MD; American Board of Surgery with subspecialty certification in surgical critical care
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.
Antiphospholipid Syndrome - Symptoms
What were your symptoms associated with antiphospholipid syndrome?Post View 27 Comments
Antiphospholipid Syndrome - Cause
What was the cause of your antiphospholipid syndrome?Post View 6 Comments
Antiphospholipid Syndrome - Effective Treatments
What kinds of treatment have been effective for your antiphospholipid syndrome?Post View 2 Comments
Antiphospholipid Syndrome - Tests
Describe the tests you've had to detect antiphospholipid antibodies and antiphospholipid syndrome.Post View 1 Comment
Top Antiphospholipid Syndrome Related Articles
acetylsalicylic acidAspirin (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
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
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 of a deep vein thrombosis in a leg are
- warmth, and
Treatment for DVT include medications and surgery.
Human Immunodeficiency Virus (HIV)The human immunodeficiency virus (HIV) causes HIV infection and the acquired immunodeficiency syndrome (AIDS). Symptoms and signs of HIV infection include fatigue, enlarged lymph glands, and recurrent vaginal yeast infections. Highly active antiretroviral therapy (ART) is the standard treatment for HIV infection.
Malaria FactsMalaria 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.
MigraineMigraine 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 Causes and SymptomsMiscarriage 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.
Signs and symptoms of pregnancy vary by stage (trimester). The earliest pregnancy symptom is typically a missed period, but others include
breast swelling and tenderness,
- nausea and sometimes vomiting,
- fatigue, and
Second trimester symptoms include
- weight gain,
- itching, and
- possible stretch marks.
Third trimester symptoms are
- additional weight gain,
- swelling of the ankles,
- fingers, and face,
- breast tenderness, and
- trouble sleeping.
Eating a healthy diet, getting a moderate amount of exercise, also are recommended for a healthy pregnancy. Information about the week by week growth of your baby in the womb are provided.
Pregnancy Planning (Preparing for Pregnancy)
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
RheumatologyRheumatology is the study of rheumatic diseases and conditions. Rheumatologists are internal medicine physicians who treat these illnesses, in particular arthritis.
Stroke Symptoms and Treatment
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
- double vision or vision loss,
- vertigo, and
- 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.
Systemic LupusSystemic 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 1A chronic inflammatory condition caused by an autoimmune disease. See a picture of Systemic Lupus Erythematosus (SLE) and learn more about the health topic.
UltrasoundUltrasound (and ultrasonography) is imaging of the body used in the medical diagnosis and screening of diseases and conditions such as:
- heart valve irregularities,
- carotid artery disease,
- heart disease,
- kidney stones,
- liver disease,
- diseases of the female reproductive, and
- diseases of the male reproductive organs.
Viral HepatitisHepatitis 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 dependant on the type of hepatitis.
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:
- Hair loss
Drug interactions, and warnings and precautions, and pregnancy and breastfeeding information should be reviewed prior to taking this medication.