Antiphospholipid Syndrome Definition, Reason, Symptoms, Treatment & Prevention
Definition
Antiphospholipid syndrome is a type of autoimmune disease, characterized by recurrent blockages in the veins or arteries, as well as recurrent miscarriages. This disease is also known as Hughes syndrome, or in layman’s sometimes called thick blood syndrome.
It is estimated that one in 2,000 people has this disease. This disease is commonly found in people with lupus, can also be found in people with strokes and heart attacks.
Antiphospholipid syndrome is more common in people aged 20–40 years and affects more women.
Antiphospholipid Syndrome Symptoms
The symptoms of the antiphospholipid syndrome can vary widely. The main symptom is a blockage of blood vessels (thrombosis). This blockage can occur in the blood vessels of the brain, coronary arteries in the heart, pulmonary arteries, blood vessels in the legs, and blood vessels in the eyes, with the following symptoms:
- Blockage in the blood vessels of the brain due to antiphospholipid syndrome causes ischemic stroke (blockage stroke) which can be characterized by sudden one-sided weakness of the arms and legs, a slanted mouth, or slurred speech.
- Blockage in the coronary arteries causes a heart attack (medically called acute coronary syndrome), characterized by sudden left chest pain such as being pressed by a heavy object, shortness of breath, or sudden death due to cardiac arrest.
- Blockage in the pulmonary arteries that occurs suddenly is called a pulmonary embolism. This disease is characterized by shortness of breath that occurs suddenly without a clear cause, and if treated too late it will cause death.
- Blockage in the leg veins can cause deep vein thrombosis (DVT). Symptoms include swelling of the lower leg (usually only on one leg), accompanied by redness and warmth of the leg.
- Blockages in the blood vessels of the eye can cause arterial or venous occlusive disease in the retina. This condition is characterized by a sharp decrease in vision that occurs suddenly.
In addition to symptoms of blocked blood vessels, antiphospholipid syndrome can also cause problems in pregnancy, in the form of:
- Recurrent miscarriage at less than 10 weeks gestation
- Miscarriage can be once or repeated, at a gestational age above 10 weeks
- Premature labor
- Fetal growth is slower than it should be
- Preeclampsia, which is a toxic condition of pregnancy characterized by hypertension and proteinuria (protein in urine) at gestational age above 20 weeks
- Eclampsia, namely preeclampsia accompanied by seizures.
Antiphospholipid Syndrome Reason
In antiphospholipid syndrome, sufferers have a type of protein called antiphospholipid antibody (aPL) in the blood. These antibodies cause blood to become easy to clot. This can lead to blockage of blood vessels (medically referred to as thrombosis) in various organs.
The cause of aPL in a person is not known clearly until now. However, it is suspected that genetic and environmental factors (such as hypertension, diabetes, obesity, exposure to cigarette smoke, pregnancy, or surgery) cause a person to be more susceptible to experiencing antiphospholipid syndrome.
In addition, there are several types of diseases that usually occur together with antiphospholipid syndromes, such as:
- Other autoimmune diseases such as lupus ( systemic lupus erythematosus / SLE), immune thrombocytopenia purpura (ITP), rheumatoid arthritis, psoriasis, Sjogren’s syndrome, or autoimmune hemolytic anemia.
- Cancers, among which are cancers of solid organs (eg liver or kidney), leukemia, lymphoma, or multiple myeloma.
- Blood disorders such as myelofibrosis, von Willebrand disease, or certain protein deficiencies (eg protein C or protein S).
- Infections such as syphilis, tuberculosis, malaria, HIV, hepatitis A, hepatitis C, varicella, measles, and certain bacterial infections.
- Nervous disease such as myasthenia gravis, multiple sclerosis or migraines.
- Side effects of certain drugs such as chlorpromazine, phenytoin, hydralazine, quinidine, clozapine, or streptomycin.
Diagnosis
To ensure the presence of antiphospholipid syndrome, several criteria must be met, namely:
- There are signs of blocked blood vessels, either in the arteries or veins, as evidenced by Dppler ultrasound examination or histopathological examination (examination to view body tissue under a microscope).
- Problems in pregnancy such as miscarriage that occurs at gestational age above 10 weeks without a clear cause, preeclampsia, eclampsia, or stunted growth of the fetus in the uterus. Or there can also be miscarriages at a gestational age of less than 10 weeks that occur repeatedly, without any chromosomal disorders in the fetus or other maternal health problems.
- Laboratory tests in the form of anticardiolipin antibodies ( anticardiolipin antibody / ACA) which show moderate or high levels. This examination must be repeated twice with a minimum distance of 6 weeks.
- Laboratory tests in the form of lupus anticoagulant antibody / LA showed positive results.
Antiphospholipid Syndrome Treatment
Treatment of antiphospholipid syndrome has several goals, namely:
- To prevent blockage of blood vessels
- Treat and remove blocked blood vessels
- Prevent complications in pregnancy
To prevent blockage of blood vessels, people with antiphospholipid syndrome will be given low-dose aspirin by a doctor. In addition, sufferers should also avoid taking hormonal contraception, stay away from exposure to cigarette smoke, and control blood pressure and cholesterol levels.
If blood vessel blockage has already occurred, then treatment with anticoagulants (a type of blood thinner) will be needed. In the early stages, heparin anticoagulants will generally be given by infusion or injection under the skin in the abdomen. After that, it will be followed by anticoagulant tablets such as warfarin or rivaroxaban.
In pregnant women who experience antiphospholipid syndrome, anticoagulants also need to be given to prevent complications in pregnancy. A safe anticoagulant for pregnant women is heparin or low molecular weight heparin (LMWH). Low-dose aspirin is also given.
Generally, drugs can prevent people with antiphospholipid syndrome from dangerous complications. But sometimes conditions can also occur that cannot be controlled with drugs.
If this is the case, surgery to install a filter (a kind of filter) in the large vein of the vena cava near the heart needs to be considered. The purpose of the filter is to filter out blood clots that can block blood vessels in various places.
Prevention
The presence of antiphospholipid syndrome cannot be prevented. However, the symptoms of blocked blood vessels and pregnancy complications caused by this syndrome can be prevented by:
- Routine control and use of drugs regularly according to doctor’s instructions
- Avoiding hormonal drugs, such as birth control pills
- Exercise regularly (4–5 times per week, for at least 30 minutes per time)
- Avoid sitting for too long (more than two hours)
- Avoid exposure to cigarette smoke
- Maintain an ideal weight