What Is Antiphospholipid Syndrome?

Antiphospholipid Syndrome or APS and also known as Hughes Syndrome is an autoimmune disorder that causes blood to clot. This condition is usually referred to and the term thick blood. Under normal conditions, antibodies play a role in fighting infection. However, in the antiphospholipid syndrome, antibodies actually act wrongly by attacking fatty compounds called phospholipids which play a role in the blood clotting process.

Symptoms of the Antiphospholipid Syndrome 
In the antiphospholipid syndrome, the immune system produces antibodies that make the blood thicker or easier to freeze than normal conditions, so it can risk causing blood clots in arteries and veins. The blood clots formed can cause APS sufferers to experience:
  • Deep vein thrombosis.
  • Pulmonary embolism.
  • Miscarriage and other pregnancy complications, such as premature birth, as well as preeclampsia and eclampsia.
  • Heart attacks and strokes, especially those that recur and occur at a younger age, namely under the age of 55 years for men, and under the age of 65 years for women.
  • Rashes and sores on the skin.
  • Blockage of blood vessels in the eyes, liver, or kidneys.
Apart from causing health problems, APS sufferers often feel symptoms and signs, such as:
  • Tingling in the arms and legs.
  • Fatigue and fatigue.
  • Recurrent headaches.
  • Impaired vision (double vision).
  • Memory disorders.
  • Talking disorders.
  • Motion and balance disorders.
  • Easy bruising due to low platelet cell counts.

Risk Factors for Antiphospholipid Syndrome 
Erroneous antibodies formed in patients with antiphospholipid syndrome (APS) tend to be more often formed in patients with the following conditions:
  • Woman.
  • Have other autoimmune diseases, such as lupus or Sjogren's syndrome.
  • Having hepatitis C infection, HIV and AIDS, or syphilis.
  • Take anticonvulsant drugs phenytoin or amoxicillin antibiotics.
  • Have a family with a history of APS.
Suffering from APS makes patients susceptible to health problems that have been mentioned, especially in circumstances, such as:
  • Is pregnant.
  • High cholesterol.
  • Lie down and sit for long periods of time, such as after surgery and long-haul flights.
  • Perform surgery, especially the leg area, such as knee or hip replacement surgery.
  • Smoke.
  • Underwent estrogen replacement therapy or consumption of birth control pills.
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Diagnosis of Antiphospholipid Syndrome 
Doctors can suspect a patient is suffering from antiphospholipid syndrome (APS) if the person has blood clots that cause the above health problems.

To prove the diagnosis of APS, the doctor will check for antibodies in the blood (antiphospholipid antibodies) that increase. The antibody consists of:
  • Anticardiolipin antibodies (ACA).
  • Lupus anticoagulant (LA).
  • Anti Beta-2 glycoprotein1 antibodies (anti B2GP1).
Examination will be repeated 12 weeks later from the previous examination, to prove that antibodies are consistently increasing.

Besides antibodies, the results of other blood tests found in APS sufferers are:
  • Decreased platelet count in blood.
  • Signs of hemolytic anemia.
  • Lengthening of freezing time activated partial thromboplastin time (aPTT).
  • Positive results on syphilis examination.
Imaging examinations such as the MRI of the brain to see strokes, as well as ultrasound of Doppler limbs to see the presence of deep vein thrombosis are needed to find problems caused by blood clots in the body.

Antiphospholipid Syndrome Treatment 
The goal of treating antiphospholipid syndrome is to prevent blood clots that can cause problems such as deep vein thrombosis, pulmonary embolism, or other problems. Reducing risk factors such as changing contraceptive options other than birth control pills and quitting smoking, is a treatment to prevent blood clots from arising. Currently low-dose aspirin or clopidogrel (if allergic to aspirin) is widely used to prevent blood clots.

If there is blood clots, the doctor will give an anticoagulant medication. Currently anticoagulant drugs that are still the choice are warfarin or heparin. Warfarin is an anticoagulant drug whose dose depends on the results of INR freezing time. The value of the INR target to be achieved is 2-3 for blood clots in veins, such as deep vein thrombosis, and 3 for blood clots in arteries, such as blood clots in the arteries of the kidney organs.

Because APS is a disease that can lead to complications in pregnancy, the selection of safe anticoagulant drugs in pregnant women is considered by doctors. For pregnant and lactating women, heparin anticoagulants, especially low molecular weight heparin (LMWH) such as enoxaparin, are injected under the skin (subcutaneous). Injections are carried out from the beginning of pregnancy up to 6 weeks after giving birth, to prevent complications in pregnancy. Aspirin can also be given during pregnancy.

The use of other types of anticoagulants in APS patients, such as rivaroxaban, is still in the research phase. Immunosuppressant drugs such as corticosteroids or rituximab can be used in patients with low platelets, wounds to the skin, or other autoimmune diseases such as lupus.

Complications of the Antiphospholipid Syndrome 
Catastrophic antiphospholipid syndrome (CAPS) is a serious complication of antiphospholipid syndrome (APS). Although rare and only occurs in 1% of APS sufferers, this complication can lead to death. In CAPS, blood clots will form throughout the body, causing multiple organ failure. It is not yet clear how these complications can form, but infections, injuries, and surgery can lead to CAPS. Symptoms that can be caused by CAPS are:
  • The fingertips turn blue.
  • Crowded.
  • Abdominal pain
  • Bloody urination.
  • Seizures.
  • Loss of consciousness.
These symptoms appear suddenly and worsen very quickly, with a high mortality rate of up to 50%.

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