An autoimmune disorder called antiphospholipid syndrome (APS) can result in blood clots and miscarriages. The body's immune system mistakenly produces antibodies that attack phospholipids, a type of fat found within all cells. There can be serious complications associated with these antibodies because they can interfere with blood vessels and the placenta.
A genetic and environmental factor may trigger APS, but its exact cause is unknown. While some people have a genetic predisposition to developing APS, others may develop it as a result of an infection, surgery, trauma, or medication. Lupus, rheumatoid arthritis, and Sjogren's syndrome can also be associated with APS.
The symptoms of APS vary depending on the location and severity of the blood clots. Some people may have no symptoms at all, while others may experience:
Blood clots in the legs (deep vein thrombosis or DVT), which can cause pain, swelling, and redness. These clots can travel to the lungs (pulmonary embolism or PE), which can cause sudden shortness of breath, chest pain, and coughing up blood.
Blood clots in the brain, which can cause stroke, transient ischemic attack (TIA), or migraine. These can cause sudden numbness, weakness, or paralysis of the face, arm, or leg, difficulty speaking or understanding speech, visual disturbances, and severe headache.
Blood clots in the heart, which can damage the heart valves and cause cardiovascular disease. This can cause chest pain, palpitations, shortness of breath, and fatigue.
Blood clots in the kidneys, spleen, or other organs, which can impair their function and cause pain, bleeding, or infection.
Recurrent miscarriages or stillbirths, especially in the second or third trimester of pregnancy. Other pregnancy complications include preeclampsia, premature delivery, and growth restriction of the baby.
Low platelet count (thrombocytopenia), which can cause bleeding from the nose, gums, or skin. The skin may also show patches of small red spots (petechiae).
Rash, which may appear as a red, lacy, net-like pattern (livedo reticularis) or as round, red spots (livedo racemosa).
It is diagnosed by a combination of clinical and laboratory criteria. Clinical criteria include having one or more episodes of blood clots or pregnancy complications. Tests for antiphospholipid antibodies must be positive on two or more occasions, at least 12 weeks apart. The most common tests are:
Anticardiolipin antibodies (aCL), which are antibodies that target cardiolipin, a phospholipid found in the inner membrane of cells.
Lupus anticoagulant (LA), which is a misnomer, as it is not an anticoagulant but rather an antibody that interferes with the clotting tests and prolongs the clotting time.
Anti-beta2-glycoprotein I antibodies (aB2GPI), which are antibodies that target beta2-glycoprotein I, a protein that binds to phospholipids and regulates the clotting process.
As part of the treatment for APS, blood thinners (anticoagulants), such as warfarin, heparin, or low molecular weight heparin, are used to prevent or treat blood clots and pregnancy complications. By inhibiting clotting factors in the blood, these medications reduce the risk of blood clots. The duration and dose of treatment depend on the severity and type of the condition. During pregnancy or for a short period of time, some people may require lifelong anticoagulation, while others may only need it during short periods of time.
Other treatments that may be used in some cases include:
Antiplatelet drugs, such as aspirin, which prevent the platelets from sticking together and forming clots.
Steroids, such as prednisone, which suppress the immune system and reduce inflammation.
Immunosuppressants, such as azathioprine, cyclophosphamide, or rituximab, which also suppress the immune system and reduce the production of antibodies.
Intravenous immunoglobulin (IVIG), which is a solution of antibodies from healthy donors that can block the harmful effects of the antiphospholipid antibodies.
Plasma exchange, which is a procedure that removes the plasma (the liquid part of the blood) and replaces it with fresh plasma or a substitute. This can lower the levels of the antiphospholipid antibodies and improve the blood flow.
There is no definitive way to prevent APS, but some measures that may help reduce the risk of blood clots and pregnancy complications include:
Avoiding smoking, as it can damage the blood vessels and increase the risk of blood clots.
Maintaining a healthy weight, as obesity can also increase the risk of blood clots.
Staying active and hydrated, as this can improve the blood circulation and prevent dehydration.
Wearing compression stockings, especially during long periods of immobility, such as travel or bed rest, as this can prevent the blood from pooling in the legs and forming clots.
Avoiding estrogen-containing contraceptives or hormone replacement therapy, as these can increase the risk of blood clots in some people with APS.
Consulting with a doctor before taking any medications or supplements that may affect the blood clotting, such as nonsteroidal anti-inflammatory drugs (NSAIDs), herbal remedies, or vitamin K.
Seeking medical attention promptly if any signs or symptoms of blood clots or pregnancy complications occur, such as pain, swelling, redness, shortness of breath, chest pain, headache, visual changes, or bleeding.
However, with proper diagnosis and treatment, most people with antiphospholipid syndrome can lead normal, productive lives.
