Anti-Phospholipid Syndrome
What is Antiphopholipid Syndrome?
Antiphospholipid syndrome in short APS, also known as Hughes syndrome or sticky blood syndrome is an auto-immune disorder whereby the body produces antiphospholipid antibodies that attack certain proteins and fats in our body namely the phospholipids. This will result in a change in the blood consistency, more stick and increase the likelihood of causing blood clots in the arteries/veins which can flow through the blood system and cause blockage to the blood supply and subsequent damage to the affected organ.
APS can cause serious complications like recurrent miscarriages, preterm labor, stroke,heart attack, deep vein thrombosis to name a few. In catastrophic anti-phospholipid syndrome, there is a "thrombotic " storm whereby multiple clots suddenly form resulting in multiple organ failure. The cause of this is unknown. This is an emergency situation with a mortality up to 46%.
There are two kinds of APS
Antiphospholipid syndrome in short APS, also known as Hughes syndrome or sticky blood syndrome is an auto-immune disorder whereby the body produces antiphospholipid antibodies that attack certain proteins and fats in our body namely the phospholipids. This will result in a change in the blood consistency, more stick and increase the likelihood of causing blood clots in the arteries/veins which can flow through the blood system and cause blockage to the blood supply and subsequent damage to the affected organ.
APS can cause serious complications like recurrent miscarriages, preterm labor, stroke,heart attack, deep vein thrombosis to name a few. In catastrophic anti-phospholipid syndrome, there is a "thrombotic " storm whereby multiple clots suddenly form resulting in multiple organ failure. The cause of this is unknown. This is an emergency situation with a mortality up to 46%.
There are two kinds of APS
- Primary antiphospholipid syndrome: It is not linked to any other disease or condition. It develops on its own
- Secondary antiphospholipid syndrome: It develops along with another autoimmune disorders such as lupus
What causes and risk factors of developing Anti-phospholipid syndrome (APS)?
The exact cause of developing APS is unknown. It is an auto-immune disease whereby the body produces antiphospholipid antibodies that attack certain proteins and fats in our body namely the phospholipids.
Risk factors for developing APS are:
The exact cause of developing APS is unknown. It is an auto-immune disease whereby the body produces antiphospholipid antibodies that attack certain proteins and fats in our body namely the phospholipids.
Risk factors for developing APS are:
- Genetic component may be a possibility hence having a family history of APS may increases your risk of getting APS
- Having a pre-existing auto-immune condition like Systemic lupus erythematosus, Sjogren's syndrome increases your risks
- Having infections like hepatitis C, HIC, cytomegalovirus and lymes disease
- Taking certain medications like hydralazine for hypertension, phenytoin for anti-seizure
Risk factors that further increase a Patient's risk of getting thrombosis (blood clots)
- Pregnancy
- Immobility : prolonged bed rest
- Oral contraceptives
- High cholesterol levels
- Smoking
- After surgery
- An associated autoimmune disease like SLE
- Atherosclerosis (Hardening of blood vessels)
Signs and symptoms of APS
The symptoms depends on where the blood clots travel to:
The symptoms depends on where the blood clots travel to:
- Complications in pregnancy: repeated miscarriages, still births, pre-term labor, pre-eclampsia (high blood pressure in pregnancy)
- Deep Vein thrombosis (DVT): blood clot forms in one of the large veins in the legs resulting in obstruction of blood flow
- Pulmonary Embolism (PE): The blood clot from the legs get dislodged and travel to the lungs resulting in tachycardia, shortness of breath, collapse and possible death.
- Ischemic attack (stroke): Blood clots moves to the brain vessels
Diagnosis of Anti-phospholipid syndrome (APS)
If a patient has symptoms of thrombosis (blood clots) in different organs or if she has recurrent pregnancy loss, then a clinical suspicion of anti-phospholipid syndrome is made. This is confirmed by doing blood tests to test the presence of the antibodies towards phospholipids. The antibodies to be tested include VDRL/RPR (syphilis test ), lupus anticoagulant, prolonged PTT, beta 2 glycoprotein I antibodies, and anticardiolipin antibody.
As recent infection and some medications may result in the presence of the antibodies in blood, so a repeated test for confirmation of APS is done after 12 weeks of the first test.
If a patient has symptoms of thrombosis (blood clots) in different organs or if she has recurrent pregnancy loss, then a clinical suspicion of anti-phospholipid syndrome is made. This is confirmed by doing blood tests to test the presence of the antibodies towards phospholipids. The antibodies to be tested include VDRL/RPR (syphilis test ), lupus anticoagulant, prolonged PTT, beta 2 glycoprotein I antibodies, and anticardiolipin antibody.
As recent infection and some medications may result in the presence of the antibodies in blood, so a repeated test for confirmation of APS is done after 12 weeks of the first test.
Treatment of APS
Treatment of APS is to reduce the tendency of blood to form clots with anti-coagulants (blood thinning medications) such as heparin,aspirin and warfarin. When you are on anti-coagulants, you will have an increased risk of bleeding. Hence your doctor will need to titrate the dosage of anti-coagulants to prevent thrombosis (blood clot) but yet minimize the side effects of bleeding with the medications.
Treatment during pregnancy also involves taking anti-coagulants like heparin or aspirin to increase chances of carry baby to term. Warfarin will cause birth defects and is avoided during pregnancy. In an unplanned pregnancy, the effectiveness of treatment may be reduced as it will not begin until several weeks after conception. If anti-coagulants are ineffective, then intravenous gamma globulin and corticosteroids like prednisolone.
By third trimester, there are no complications then heparin can be stopped but aspirin should be continued till delivery.
Treatment for catastrophic anti-phospholipid syndrome:
In catastrophic anti-phospholipid syndrome, there is a "thrombotic " storm whereby multiple clots suddenly form resulting in multiple organ failure. The cause of this is unknown. This is an emergency situation with a mortality up to 46%. Symptoms depend on which organs are affected , some patients may experience:
- confusion
- abdomen pain
- fits and seizures
- coma
- breathlessness
- swelling (edema) in limbs
- death in serious cases.
Patients with catastrophic anti-phospholipid syndrome must be admitted into Intensive Care unit for organs support. The will be put on high dosage of anti-coagulants, corticosteroids and plasma exchange (plasmapheresis). About half may not survive the first episode and there is a risk of a recurrent episode.
Treatment of APS is to reduce the tendency of blood to form clots with anti-coagulants (blood thinning medications) such as heparin,aspirin and warfarin. When you are on anti-coagulants, you will have an increased risk of bleeding. Hence your doctor will need to titrate the dosage of anti-coagulants to prevent thrombosis (blood clot) but yet minimize the side effects of bleeding with the medications.
Treatment during pregnancy also involves taking anti-coagulants like heparin or aspirin to increase chances of carry baby to term. Warfarin will cause birth defects and is avoided during pregnancy. In an unplanned pregnancy, the effectiveness of treatment may be reduced as it will not begin until several weeks after conception. If anti-coagulants are ineffective, then intravenous gamma globulin and corticosteroids like prednisolone.
By third trimester, there are no complications then heparin can be stopped but aspirin should be continued till delivery.
Treatment for catastrophic anti-phospholipid syndrome:
In catastrophic anti-phospholipid syndrome, there is a "thrombotic " storm whereby multiple clots suddenly form resulting in multiple organ failure. The cause of this is unknown. This is an emergency situation with a mortality up to 46%. Symptoms depend on which organs are affected , some patients may experience:
- confusion
- abdomen pain
- fits and seizures
- coma
- breathlessness
- swelling (edema) in limbs
- death in serious cases.
Patients with catastrophic anti-phospholipid syndrome must be admitted into Intensive Care unit for organs support. The will be put on high dosage of anti-coagulants, corticosteroids and plasma exchange (plasmapheresis). About half may not survive the first episode and there is a risk of a recurrent episode.
Prevention of APS complications
The main aim is to prevent and lower the risk of blood clots :
- Quit smoking
- Exercise regularly do not stay immobile
- Maintaining a healthy body weight
- have good control of cardiovascular diseases i.e. hypertension , hyperlipidemia and diabetes.
- Limit food rich in vitamin K and other medications that may have drug interactions with warfarin which patients are already taking.
The main aim is to prevent and lower the risk of blood clots :
- Quit smoking
- Exercise regularly do not stay immobile
- Maintaining a healthy body weight
- have good control of cardiovascular diseases i.e. hypertension , hyperlipidemia and diabetes.
- Limit food rich in vitamin K and other medications that may have drug interactions with warfarin which patients are already taking.