Antiphospholipid Antibody Syndrome, also known as Hughes or just SAF or SAAF, is a rare autoimmune disease characterized by the ease in forming thrombi in the veins and arteries that interfere with blood clotting, which can result in headache, difficulty breathing and heart attack, for example.
According to the cause, SAF can be classified into three main types:
- Primary, in which there is no specific cause;
- Secondary, which happens as a consequence of another disease, and is usually related to Systemic Lupus Erythematosus. Secondary APS can also happen, although it is more rare, associated with other autoimmune diseases, such as scleroderma and rheumatoid arthritis, for example;
- Catastrophic, which is the most severe type of APS in which thrombi are formed in at least 3 different locations in less than 1 week.
APS can happen at any age and in both sexes, however it is more frequent in women between 20 and 50 years old. Treatment must be established by the general practitioner or rheumatologist and aims to prevent the formation of thrombi and prevent complications, especially when the woman is pregnant.
Main signs and symptoms
The main signs and symptoms of APS are related to changes in the coagulation process and the occurrence of thrombosis, the main ones being:
- Chest pain;
- Difficulty breathing;
- Headache;
- Nausea;
- Swelling of the upper or lower limbs;
- Decrease in the amount of platelets;
- Successive spontaneous abortions or changes in the placenta, with no apparent cause.
In addition, people diagnosed with APS are more likely to have kidney problems, heart attack or stroke, for example, due to the formation of thrombi that interfere with blood circulation, changing the amount of blood that reaches the organs. Understand what thrombosis is.
What causes the syndrome
Antiphospholipid Antibody Syndrome is an autoimmune condition, which means that the immune system itself attacks cells in the body. In this case, the body produces antiphospholipid antibodies that attack the phospholipids present in fat cells, which makes it easier for the blood to coagulate and form thrombi.
The specific reason why the immune system produces this type of antibody is not yet known, but it is known that it is a more frequent condition in people with other autoimmune diseases, such as Lupus, for example.
How the diagnosis is made
The diagnosis of Antiphospholipid Antibody Syndrome is defined by the presence of at least one clinical and laboratory criteria, that is, the presence of a symptom of the disease and the detection of at least one autoantibody in the blood.
Among the clinical criteria considered by the doctor are episodes of arterial or venous thrombosis, occurrence of abortion, premature birth, autoimmune diseases and the presence of risk factors for thrombosis. These clinical criteria must be proven through imaging or laboratory tests.
Regarding laboratory criteria are the presence of at least one type of antiphospholipid antibody, such as:
- Lupus anticoagulant (AL);
- Anticardiolipin;
- Anti beta2-glycoprotein 1.
These antibodies must be evaluated at two different times, with an interval of at least 2 months.
For the diagnosis to be positive for APS, it is necessary that both criteria are confirmed through examinations performed twice with an interval of at least 3 months.
How the treatment is done
Although there is no treatment capable of curing APS, it is possible to reduce the risk of clot formation and, consequently, the appearance of complications such as thrombosis or infarction, through the frequent use of anticoagulant drugs, such as Warfarin, which is for oral use , or Heparin, which is for intravenous use.
Most of the time, people with APS who are undergoing treatment with anticoagulants are able to lead a completely normal life, it is only important to have regular appointments with the doctor to adjust the doses of medications, whenever necessary.
However, to ensure the success of the treatment, it is still important to avoid some behaviors that may impair the effects of anticoagulants, as is the case of eating foods with vitamin K, such as spinach, cabbage or broccoli, for example. Check out other precautions you should take when using anticoagulants.
Treatment during pregnancy
In some more specific cases, such as during pregnancy, the doctor may recommend that the treatment be done with injectable Heparin associated with Aspirin or an intravenous Immunoglobulin, to prevent the occurrence of complications such as abortion, for example.
With proper treatment, there are great chances that the pregnant woman with APS will have a normal pregnancy, however it is necessary that she is closely monitored by the obstetrician, since she is at greater risk of miscarriage, premature birth or pre-eclampsia. Learn how to recognize the symptoms of preeclampsia.
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Bibliography
- NHS. Antiphospholipid syndrome (APS). Available in: . Accessed on 24 Apr 2019
- MEDSCAPE. Antiphospholipid Syndrome. Available in: . Accessed on 24 Apr 2019
- BRAZILIAN SOCIETY OF RHEUMATOLOGY. Antiphospholipid Syndrome. Available in: . Accessed on 24 Apr 2019
- GOLDMAN, Lee; SCHAFER, Andrew I .. Goldman-Cecil Medicine. 25 ed. Rio de Janeiro: Elsevier, 2018. p. 1198-1199.
