Antiphospholipid syndrome or antiphospholipid syndrome ( APS) is a group of symptoms that occur as a result of the body's immune system attacking fatty compounds in the body called phospholipids. The most characteristic sign of antiphospholipid syndrome is increased blood viscosity .
Antiphospholipid syndrome is also known as Hughes syndrome, after its discoverer. This syndrome is classified as an autoimmune disease that can cause disturbances in all parts of the body.
Phospholipids are body fat compounds that build all cell walls in the human body. Phospholipids also play a major role in the process of blood clotting by platelets . Therefore, blood clots are one of the most common signs of this condition.
Causes of Antiphospholipid Syndrome
In antiphospholipid syndrome, the body's immune system (immune system) which is supposed to fight foreign organisms, such as viruses or bacteria, mistakenly produces antibodies that attack phospholipids.
What causes these antibodies to form or how these antibodies cause blood clots is not known for certain. However, it is suspected that these antibodies are formed due to genetic mutations in the immune system, certain viral or bacterial infections, certain medications, or a combination of the three.
There are several factors that can increase a person's risk of developing antiphospholipid syndrome, namely:
- Female gender
- Suffer from another autoimmune disease, such as lupus or Sjögren's syndrome
- Suffering from certain infections, such as hepatitis C, HIV/AIDS, or syphilis
- Taking certain drugs, such as the anticonvulsant phenytoin or the antibiotic amoxicillin
- Have a family history of antiphospholipid syndrome
Recent studies have also revealed the discovery of antibodies that attack phospholipids in COVID-19 patients . This is thought to be related to blood clots that occur in COVID-19 patients with severe symptoms. However, this still needs further research.
In some cases, a person may have antibodies that attack phospholipids in their blood without experiencing any health problems. Even so, people with these conditions will still be at risk of experiencing symptoms if:
- Currently in pregnancy
- Have high blood cholesterol levels
- Undergoing surgery, especially on the legs, such as knee or hip joint replacement surgery
- Have a smoking habit
- Undergoing hormone replacement therapy or taking birth control pills
- Are overweight or obese
- Not moving for a long time, for example due to being on bed rest after surgery or sitting during long haul flights
Symptoms of Antiphospholipid Syndrome
Antiphospholipid syndrome causes blood to become thicker or clot more easily. This increases the risk of blockage of blood flow in the arteries and veins.
Blood clots that form can cause APS sufferers to experience:
- Deep vein thrombosis (DVT) or deep vein thrombosis
- Pulmonary embolism
- Rashes or sores on the skin
- Heart attacks or strokes , especially those that are repeated and occur at the age of under 55 for men and under the age of 65 for women
- Blockage of blood vessels in the eyes, liver, or kidneys
- Pregnancy complications, such as recurrent miscarriage or premature birth caused by severe preeclampsia or eclampsia
In addition, antiphospholipid syndrome is also known to cause heart valve disorders, nervous system disorders, and thrombocytopenia .
The above conditions can be recognized by symptoms, such as:
- Frequent tingling in the feet or hands
- Fatigue and limp
- Recurrent headaches
- Visual disturbances, such as double vision
- Memory impairment
- Speech disorder
- Movement and balance disorders
- Bruises or cuts on the skin
- Nosebleeds and bleeding gums
When to see a doctor
Check with your doctor if you experience the above symptoms for no apparent reason, especially if the symptoms occur frequently and are quite disturbing.
Immediately visit the emergency room or the nearest doctor if you suddenly experience an emergency health problem, such as:
- Stroke, which is characterized by severe headaches, muscle weakness or numbness on one side of the body, difficulty speaking, or difficulty understanding other people's words
- Pulmonary embolism, which is characterized by shortness of breath, chest pain when inhaling, and coughing up blood
- Deep vein thrombosis , which is characterized by swelling, redness, and pain in the calf or arm
Diagnosis of Antiphospholipid Syndrome
To diagnose antiphospholipid syndrome or APS, the doctor will ask questions and answers about the symptoms experienced by the patient, history of the patient's and family's health conditions, and the drugs currently being used. After that, the doctor will carry out a thorough physical examination.
If the patient has blood clots that cause the health problems mentioned above and without a clear cause or risk factor, the doctor will do a blood test to confirm the presence of antibodies that cause APS.
Blood tests will be carried out 2 times. Patients can be diagnosed with antiphospholipid syndrome if antibodies that cause APS are found in both tests.
In addition to antibody testing, doctors can also perform the following tests to make the diagnosis more accurate:
- General check up
- Syphilis test
- Blood clotting test
- Examination of lupus antibodies and Anti-beta-2 glycoprotein I
Radiological examinations are also needed to detect problems that may occur due to blood clots in the body, such as an MRI of the brain to see a stroke, or a Doppler ultrasound of the legs to see the presence of deep vein thrombosis (DVT).
Antiphospholipid Syndrome Treatment
There are several methods that can be used to treat antiphospholipid syndrome, namely:
Prevention of blood clots
Because they have a tendency to develop blood clots, APS sufferers need to regularly take blood-thinning drugs, such as low-dose aspirin or clopidogrel , to prevent this from happening. If you take birth control pills, APS sufferers will be advised to replace it with another method of contraception, for example an IUD .
In addition, the risk of blood clots can also be reduced by making lifestyle changes, such as:
- Maintain an ideal weight
- Limit foods high in fat and sugar
- Exercise regularly
- Stop smoking
- Avoid consuming alcohol
Treatment of blood clots
If APS sufferers have had blood clots before, the doctor will give anticoagulant drugs , such as warfarin , in the form of drinking tablets. However, if symptoms of blood clots suddenly become severe, people with APS need to get injectable anticoagulants, such as heparin .
Treatment in pregnancy
Treatment or prevention of blood clots in pregnant women with APS syndrome is generally carried out by administering a combination of injectable heparin and low-dose aspirin. However, the dosage and time of administration will vary, depending on the risk factors that pregnant women have.
In addition to the above treatment methods, immunosuppressant drugs, such as corticosteroids or rituximab, can also be used to treat APS syndrome in patients with low platelets (thrombocytopenia), have skin wounds, or have another autoimmune disease, such as lupus.
Complications of the Antiphospholipid Syndrome
Catastrophic antiphospholipid syndrome (CAPS) is a serious complication of antiphospholipid syndrome (APS). Even though it only occurs in 1% of APS sufferers, this complication needs to be watched out for because it can result in death.
In CAPS, blood clots form all over the body, causing organ failure to occur at the same time. It is not clear how this complication occurs, but it is suspected that the triggers are infection, injury, and surgery.
CAPS can be recognized by the following symptoms:
- Fingertips turn blue
- Crowded
- Abdominal pain
- Pass bloody urine
- seizures
- Loss of consciousness
These symptoms usually appear suddenly and get worse very quickly.
Antiphospholipid Syndrome Prevention
Antiphospholipid syndrome is a type of disease that is difficult to prevent because the exact cause is unknown. The best prevention effort that can be done is to avoid factors that can increase the risk of this disease.
Carrying out routine health checks before there are any complaints can also be a good prevention for people with autoimmune diseases or people who have a family history of autoimmune diseases or antiphospholipid syndrome.