Table of contents:
- Main signs and symptoms
- What causes the syndrome
- How the diagnosis is made
- How the treatment is done
Antiphospholipid Antibody Syndrome, also known as Hughes syndrome or just APS or APS, is a rare autoimmune disease characterized by the ease of forming thrombi in veins and arteries that interfere with blood clotting, which can result in headache, difficulty breathing and heart attack, for example.
According to the cause, FAS can be classified into three main types:
- Primary, where there is no specific cause;
- Secondary, which occurs as a consequence of another disease, and is usually related to Systemic Lupus Erythematosus. Secondary FAS can also happen, although it is rarer, associated with other autoimmune diseases, such as scleroderma and rheumatoid arthritis, for example;
- Catastrophic, which is the most severe type of APS in which thrombus forms in at least 3 different sites in less than 1 week.
FAS can happen at any age and in both sexes, however it is more common in women between 20 and 50 years old. Treatment must be established by the general practitioner or rheumatologist and aims to prevent thrombus formation and avoid complications, especially when the woman is pregnant.
Main signs and symptoms
The main signs and symptoms of FAS are related to changes in the clotting process and the occurrence of thrombosis, the main ones being:
- Chest pain;
- Difficulty breathing;
- Swelling of the upper or lower limbs;
- Decrease in the number of platelets;
- Successive spontaneous abortions or changes in the placenta, with no apparent cause.
In addition, people diagnosed with FAS are more likely to have kidney problems, heart attack or stroke, for example, due to the formation of thrombi that interfere with blood circulation, altering the amount of blood arriving in 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 the body's cells. In this case, the body produces antiphospholipid antibodies that attack the phospholipids present in fat cells, which makes it easier for the blood to clot and form thrombi.
The specific reason that leads the immune system to produce this type of antibodies 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 other 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 physician are episodes of arterial or venous thrombosis, occurrence of miscarriage, premature birth, autoimmune diseases and presence of risk factors for thrombosis. These clinical criteria must be confirmed by imaging or laboratory tests.
Regarding laboratory criteria are the presence of at least one type of antiphospholipid antibody, such as:
- Lupus anticoagulant (AL);
- Anti beta2-glycoprotein 1.
These antibodies should be evaluated at two different times, with an interval of at least 2 months.
For the diagnosis to be positive for FAS, both criteria must be confirmed by means of tests performed twice with an interval of at least 3 months.
How the treatment is done
Although there is no treatment capable of curing FAS, it is possible to reduce the risk of clot formation and, consequently, the emergence 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 FAS who are being treated with anticoagulants manage to have a completely normal life, and it is just important to maintain regular consultations with the doctor to adjust the doses of medication, 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, such as the ingestion of 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 with an intravenous Immunoglobulin, to prevent the occurrence of complications such as miscarriage, for example.
With proper treatment, there is a high chance that a pregnant woman with FAS will have a normal pregnancy, however, she must be closely monitored by the obstetrician, since she has a higher risk of miscarriage, premature birth or preeclampsia. Learn how to recognize the symptoms of preeclampsia.