Antiphospholipid Syndrome (Hughe’s syndrome)

Antiphospholipid Syndrome (Hughe’s syndrome)

Antiphospholipid syndrome is an autoimmune disorder characterized by the formation of blood clots in your arteries, veins and organs. In antiphospholipid syndrome, antibodies are produced against proteins that bind phospholipids. The antibodies are of 3 types: anticardiolipin antibodies, the lupus anticoagulant and antibodies directed against specific molecules including a molecule known as beta-2-glycoprotein 1. Phospholipids are a type of fat that plays a key role in clotting. When antibodies attack the phospholipid-binding proteins, it causes blood to clot abnormally.

Causative and risk factors

Antiphospholipid syndrome is of 2 types:

  • Primary: When there is no known underlying reason
  • Secondary: When there is an underlying condition or medication

Risk factors for developing antiphospholipid syndrome include having underlying autoimmune or infectious conditions. Commonly found underlying autoimmune conditions include SLE or Sjogren’s syndrome. Commonly seen underlying infections include syphilis, HIV, hepatitis C or Lyme disease. Taking certain medications, such as hydralazine, quinidine, phenytoin and amoxicillin can also trigger antiphospholipid syndrome. Having a family member with antiphospholipid syndrome is associated with a higher risk of developing it.

In some individuals, antibodies associated with antiphospholipid syndrome are present without developing signs or symptoms. However, if you have these antibodies, your risk of developing blood clots increases particularly during periods of pregnancy, immobility or surgery. Individuals, who smoke, take oral contraceptives or have high cholesterol and triglycerides levels are at a higher risk.

 

Clinical presentation

Antiphospholipid syndrome can give rise to blood clots which may lead to deep vein thrombosis, peripheral arterial thrombosis or pulmonary embolism.

It can give rise to certain complications during pregnancy such as repeated miscarriages, stillbirths, premature delivery and pre-eclampsia. Thrombocytopenia may occur, giving rise to petechiae and episodes of bleeding.

Neurological symptoms such as chronic headaches, dementia and seizures may occur. Other symptoms that may be present include stroke, damaged heart valves, chorea and cognitive problems.

 

Diagnosis/Tests  

Blood tests are performed to detect certain antibodies like lupus anticoagulant, anti-cardiolipin and beta-2 glycoprotein I.

 

Treatment  

Antiphospholipid syndrome cannot be cured but treatment is available to reduce the complications. Anticoagulant medication like heparin, warfarin and aspirin are to continued lifetime. Special monitoring is necessary in pregnant women to prevent complications.

Antiphospholipid syndrome, if left untreated, can lead to permanent organ damage or death.

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