Antiphospholipid (Hughes) Syndrome

Antiphospholipid syndrome, also known as Hughes Syndrome, is an autoimmune disorder which causes an increased risk of blood clots. People with this condition are at an increased risk of developing:shutterstock_53224042_height-400.jpg

  • Deep vein thrombosis
  • Arterial thrombosis
  • Blood clots in the brain
  • Pregnant women have an increased risk of miscarriage or stillbirth

What causes antiphospholipid syndrome? 

With antiphospholipid syndrome the immune system attacks healthy tissue; abnormal antibodies are produced which target proteins attached to fat molecules, making the blood more likely to clot.

Diagnosing antiphospholipid syndrome

Blood tests are used to diagnose antiphospholipid syndrome; these tests look for antibodies responsible antiphospholipid syndrome.

How antiphospholipid syndrome is treated

Antiphospholipid syndrome cannot be cured; however, it can be managed successfully. Blood thinning injections, such as fragmin can be self-administered and aspirin can also be taken to help thin the blood. These medications can also improve a pregnant woman’s chance of having a successful preEIS05000.jpggnancy.

Pregnancy 

Antiphospholipid syndrome can cause recurrent miscarriage or stillbirth, as well as other pregnancy complications. At least 15% of recurrent miscarriages occur as a result of antiphospholipid syndrome, and with prompt treatment, the pregnancy success rate has risen from 20% before 1990 to over 80% today.

Pregnant ladies are usually treated daily with low dose (75mg) aspirin, and if a previous loss has occurred in the second or third trimesters they may also be given fragmin injections.

Pregnancies can be affected in a number of ways:

Early pregnancy loss

Most miscarriages occur during the first 13 weeks; antiphospholipid antibodies can cause early miscarriages by preventing the embryo from embedding properly in the womb. Early miscarriages are common, and there are many possible causes. Therefore, women will not be tested for antiphospholipid antibodies until they have three consecutive early miscarriages.

Late pregnancy loss

In most pregnancies foetal death in the second and third trimesters is rare; however, it is strongly associated with antiphospholipid syndrome and therefore women with a late pregnancy loss should be tested for antiphospholipid antibodies. Women with antiphospholipid syndrome can develop clots in the placenta or around the cord which reduces the baby’s oxygen supply.

Pre-eclampsia 

Pre-eclampsia is twice as likely to occur in women with antiphospholipid syndrome.

Intrauterine growth restriction 

Intrauterine growth restriction (IUGR) are babies with a very low birthweight and they usually weigh less than 90% of babies at the same gestational age. With antiphospholipid syndrome the reduced blood flow to the placenta can cause the baby to be small for dates.

Research

What is being done to treat Antiphospholipid (Hughes) Syndrome? And what research is in place to find things to help someone with this condition? I recently came across ‘The London Bridge Hospital’ website, they are a private hospital which specialises in treating Lupus and Hughes syndrome as well as conducting clinical research.

From The London Bridge Hospital website ‘In 1983, our group described a condition in which there was an increased tendency to blood clotting. Such clots can occur at any time, and can affect veins or arteries anywhere in the body. During pregnancy, clotting of the placenta results in a tendency to miscarriage – some women suffering a dozen or more miscarriages before correct diagnosis and treatment. Features include leg and arm clots (DVTs), headache and migraine (leading in some to stroke), memory loss, chest pain, cold circulation. We initially reported the condition in a group of lupus patients but soon recognised that it could occur in individuals without any evidence of lupus’

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Clinical Studies for Antiphospholipid (Hughes) Syndrome Research

Units throughout the world are researching Antiphospholipid Syndrome; The unit at St Thomas’ obtains some funding from the Hughes Syndrome Foundation and publishes up to 40 research papers each yeaaps3r; and The London Bridge hospital is now carrying out studies with groups in America, Argentina, Brazil, France, Italy, Japan, Portugal and Spain. The International Antiphospholipid Syndrome meeting (Dr Hughes first started this in 1985) is now in its 12th meeting, and it attracts up to 1000 doctors and researchers. The discovery of Antiphospholipid Syndrome in obstetrics has meant that the odds of carry a baby to term has increased dramatically; before the discovery patients only had a 20% chance of a successful pregnancy but treatment patients now have an 80% chance.

General treatments

The aim of treatment is to ‘thin’ the bloody so that the tendency to clot is reduced, this is achieved with anticoagulant medication. There are currently three main anticoagulant medications (aspirin, heparin or warfarin) and for most patients one low dose aspirin (75mg) a day is enough to stop the blood from clotting. Heparin (such as Fragmin) is a very effective anticoagulant and is offered in injection form. It cannot be used for long term treatment due to an increased risk of osteoporosis. It is, however, used in some Antiphospholipid Syndrome pregnancies, as warfarin is potentially toxic to the developing baby.

Aspirin 

  • aps5Low dose aspirin (baby aspirin) – at a dose of between 75mg to 100mg a makes the blood platelets less sticky.
  • Side effects are rare (indigestion; allergy especially in asthmatics)
  • Safe in pregnancy

Heparin

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  • Newer “low molecular weight” heparin (such as Fragmin) has replaced older preparations
  • Only available as injection
  • Used to treat thrombosis, prior to warfarin use
  • Safe in pregnancy

Warfarin (Coumadin)

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  • Warfarin is a safe and effective medicine; despite being dismissed as ‘rat poison’ by some journalists
  • Side effects are rare – the only vital issue is to keep the dosage correct
  • The thinning of the blood achieved by warfarin is measured on an ‘International’ scale called ‘INR’ ‘Normal’ is “1” – half thick blood is “2” and one third blood is “3”
  • Not allowed in pregnancy

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