About antiphospholipid syndrome

What is antiphospholipid syndrome?

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 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 (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.

What are the symptoms for antiphospholipid syndrome?

Signs and symptoms of antiphospholipid syndrome can include:

  • Blood clots in your legs (DVT). The clots can travel to your lungs (pulmonary embolism).
  • Repeated miscarriages or stillbirths. Other complications of pregnancy include premature delivery and high blood pressure during pregnancy (preeclampsia).
  • Stroke. A stroke can occur in a young person who has antiphospholipid syndrome but no known risk factors for cardiovascular diseases.
  • Transitory ischemic attack (TIA). Similar to a stroke, a TIA usually lasts only a few minutes and causes no permanent damage.
  • Rash. Some people develop a red Rash with a lacy, net-like pattern (livedo reticularis).

Less common signs and symptoms include:

  • Neurological symptoms. Chronic Headaches, including migraines; dementia and Seizures are possible when a blood clot blocks blood flow to parts of your brain.
  • Cardiovascular disease. Antiphospholipid syndrome can damage heart valves.
  • Bleeding. Some people have a decrease in blood cells needed for clotting (platelets). If you have this condition (thrombocytopenia), you might have few or no symptoms.

    However, if your platelet count drops too low, you might have episodes of bleeding, particularly from your nose and gums. You can also bleed into your skin, which will appear as patches of small red spots (petechiae).

When to see a doctor

If you have another autoimmune condition, talk to your doctor about whether you should be tested for antiphospholipid antibodies.

Other reasons to contact your doctor include:

  • Pain, Swelling, redness, or tenderness in your leg or arm. Seek emergency care if vein Swelling and pain are severe or are accompanied by Chest pain or Shortness of breath.
  • Vaginal spotting or bleeding during pregnancy. This can be a sign of miscarriage or other pregnancy problems. However, many women who spot or bleed have a healthy pregnancy.

    If you've had pregnancy losses or unexplained severe complications of pregnancy, talk to your doctor about getting tested.

If you have antiphospholipid syndrome and you're thinking about getting pregnant, ask your doctor what treatments are available during your pregnancy.

When it's an emergency

Seek emergency care if you have signs and symptoms of:

  • Stroke. These include sudden numbness, Weakness or paralysis of your face, arm or leg; difficulty speaking or understanding speech; visual disturbances; severe Headache; and Dizziness
  • Pulmonary embolism. These include sudden Shortness of breath, Chest pain and coughing up blood-streaked mucus
  • Deep vein thrombosis. These include leg Swelling or pain
  • Other bleeding. These include unexplained bleeding from your nose or gums; an unusually heavy menstrual period; vomit that is bright red or looks like coffee grounds; , tarry stool or bright red stool; and unexplained abdominal pain

What are the causes for antiphospholipid syndrome?

The role of phospholipids

In antiphospholipid syndrome, your blood clots abnormally because your body mistakenly produces antibodies that attack phospholipids, a type of fat that plays a key role in clotting. Antibodies are proteins that normally protect the body against invaders, such as viruses and bacteria.

You can have antiphospholipid antibodies, but you'll be diagnosed with the syndrome only if they cause health problems. Antiphospholipid syndrome can be caused by an underlying condition, such as an autoimmune disorder, infection or certain medications, or you can develop the syndrome without an underlying cause.

What are the treatments 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.

What are the risk factors for antiphospholipid syndrome?

Antiphospholipid syndrome affects women much more than it does men. Other risk factors include:

  • Having an autoimmune condition, such as systemic lupus erythematosus or Sjogren's syndrome
  • Having certain infections, such as syphilis, HIV/AIDS, hepatitis C or Lyme disease
  • Taking certain medications, such as hydralazine for high blood pressure, the heart rhythm-regulating medication quinidine, the anti-seizure medication phenytoin (Dilantin) and the antibiotic amoxicillin
  • Having a family member with antiphospholipid syndrome

Risk factors for developing symptoms

It's possible to have the antibodies associated with antiphospholipid syndrome without developing signs or symptoms. However, having these antibodies increases your risk of developing blood clots, particularly if you:

  • Become pregnant
  • Are immobile for a time, such as being on bed rest or sitting during a long flight
  • Have surgery
  • Smoke cigarettes
  • Take oral contraceptives or estrogen therapy for menopause
  • Have high cholesterol and triglycerides levels

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