Antiphospholipid Syndrome


It is an autoimmune disease where antiphospholipid antibodies are produced against the body’s own phospholipid present in the cell membrane or towards protein bound to phospholipids. It is more prevalent in young women and also known as Hughes syndrome and there is even a risk of blood clots and miscarriages in pregnant women. It can be primary or secondary

  • PRIMARY: occurs on its own.
  • SECONDARY: is associated with other autoimmune diseases such as SLE.

CAUSES:

The cause is due to genetic and environmental factors. HLA-DR7 gene encodes major histocompatibility complex MHC CLASS III sits on the surface of the cell and they help activate B cell and starts producing antibodies. If this gene HLA-DR7 gets mutated it starts producing autoantibodies against phospholipids. Other triggers for antiphospholipid syndrome include infections such as HIV, syphilis, hepatitis C, malaria and certain drugs such as procainamide, cardiovascular drugs, quinidine, propranolol, few antipsychotic drugs. Two antiphospholipid antibodies that are produced are :

  • Anti-beta-2 glycoprotein 1- which causes blood clots by agglutination.
  • Anti-cardiolipin – that targets cardiolipin 

Antiphospholipid antibodies can cause deep vein thrombosis in women and blood clots and arterial thrombosis which is seen mainly in males.

SYMPTOMS:

  • DEEP VEIN THROMBOSIS:  causes clot formation in the legs and arms and can cause pulmonary embolism.
  • PREGNANCY COMPLICATIONS: includes recurring miscarriages, high blood pressure, placental infarction.
  • RENAL FAILURE 
  • LIBIDO VERTICULARIS: swelling of the venules due to clots.
  • ISCHAEMIC STROKE: due to arterial thrombosis.

DIAGNOSIS:

  • Complete medical and personal history of the patient by the doctor.
  • Blood test
  • Lupus anticoagulant assay

TREATMENT:

  • Avoiding risk factors like smoking and oral contraceptives.
  • Aspirin is an antiplatelet drug taken to prevent thrombosis.
  • Warfarin is an anticoagulant drug taken to prevent blood clotting but during pregnancy, warfarin can cross the placenta to cause toxicity, so it should be replaced with low molecular weight warfarin until 6 weeks after delivery.
  • Corticosteroids should be given to suppress the immune system for underlying associated autoimmune diseases.

 

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