Antiphospholipid syndrome
What is antiphospholipid syndrome (APS)?
Antiphospholipid syndrome (APS) is also known as 'sticky blood
syndrome' or Hughes Syndrome after the doctor who discovered the
condition in the early 1980s.
APS
can cause blood clotting in
the arteries or veins and is also a major cause of recurrent
miscarriage.
It affects all age groups but is most common between the ages of
20 and 50.
APS
can
occur either on its own or alongside lupus.
In lupus the immune system goes into overdrive and produces a
huge variety of excess
antibodies. One type,
antiphospholipid antibodies, is associated with blood
clotting. We now know that antiphospholipid antibodies can also
exist in people who don't have lupus. Clotting can affect any vein
or artery in the body, resulting in a variety of symptoms.
It is estimated that 1 in 5 people who have had a stroke before
the age of 40 may have
APS
.
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Antiphospholipid syndrome symptoms
The two main problems caused by antiphospholipid syndrome are
blood clotting and pregnancy problems, particularly recurrent
miscarriage.
Blood clotting
(thrombosis)
can occur:
- in the veins, causing pain and swelling, typically in the calf
(
deep
vein thrombosis
or DVT) – this can sometimes lead to
pulmonary embolism
if a piece of the clot breaks away and
travels to the lung
- in the arteries, causing high blood-pressure or strokes
- in the brain, which can cause memory loss, migraines,
forgetfulness, slurred speech, fits or visual disturbances.
In pregnancy, antiphospholipid syndrome can cause recurrent
miscarriage. This is because the increased 'stickiness' of the
blood means it is unable to pass through the smallest blood vessels
in the afterbirth (placenta). This can happen at any time during
the pregnancy but is most common between three and six months.
APS
can also cause
other pregnancy complications such as high blood pressure
(pre-eclampsia), small babies and early deliveries.
Other problems sometimes associated with antiphospholipid
syndrome include:
- Heart problems – The heart valves may thicken
and fail to work, or the arteries may thicken, leading to
angina.
- Kidney problems –
APS
can cause narrowing of the
blood vessels, resulting in high blood pressure.
- Infertility – Testing for antiphospholipid
antibodies is becoming routine in infertility clinics.
- Skin problems – Some people develop a blotchy
rash, often seen on the knees or wrists, with a lacy pattern
(livedo
reticularis).
- A low platelet count – A reduction in one of
the white blood cells (platelets) is also found in some people with
APS
. Often there
aren't any symptoms of this, although people with very low counts
may bruise easily or experience abnormal or excessive
bleeding.
How is antiphospholipid syndrome diagnosed?
There are two main blood tests used to diagnose antiphospholipid
syndrome:
- the
anticardiolipin test
- the lupus
anticoagulant test.
Although these tests measure broadly the same thing, around 20
per cent of people with antiphospholipid syndrome will have a
negative result in one test or the other, so one test alone could
miss the diagnosis. Similarly, a single positive test may not be
significant, especially if it's only just positive.
The tests are usually repeated after 6–8 weeks as levels of
antiphospholipid antibodies vary and can sometimes be associated
with infections or use of antibiotics.
Higher levels of antibodies suggest you may be at greater risk
of blood clots and other symptoms, but even if you do test positive
it doesn't mean that you will definitely have any of the problems
described.
A past medical history (e.g. migraine, recurrent miscarriages)
sometimes helps to make the diagnosis of APS.
Antiphospholipid syndrome treatments
At present antiphospholipid syndrome can't be cured but the
effects can be controlled. Treatment with anticoagulant
(blood-thinning) drugs can help prevent both blood clots and
miscarriages.
The most commonly used drugs are aspirin, warfarin and
heparin.
- If you have antiphospholipid antibodies but no history of
clotting, your doctor will probably recommend low-dose aspirin
(75–100 mg) daily. This is not guaranteed to prevent blood clots,
but is known to make the blood less 'sticky'. Research is currently
comparing the use of aspirin with very low-dose warfarin.
- If you have APS and a history of clotting you're likely to be
given warfarin to prevent further blood clots. Warfarin is taken by
mouth. You will have regular blood tests to check what effect it is
having – if necessary the dose will be adjusted.
- If you've had a number of miscarriages, but no history of
clotting, the usual treatment is low-dose aspirin. However,
injections of heparin are increasingly common, especially if the
previous miscarriages happened in mid- to late pregnancy, or if
there have been other pregnancy complications such as
pre-eclampsia.
- If you are on
warfarin and you become pregnant you'll probably be
changed over to heparin. This
is because warfarin is potentially toxic to the baby.
Even with treatment, complications can sometimes occur towards
the end of pregnancy. However, advances in the understanding and
treatment of APS have resulted in many more successful pregnancies
in women with APS.
With close monitoring of the pregnancy, there is now a very good
chance that your baby will do very well with no long-term
problems.
Self-help and daily living
It's been suggested that increasing the amount of essential
fatty acids in your diet, particularly omega-3 fatty acids found in
oily fish, should help reduce the risk of thrombosis.
However, there are no clinical trials to support this idea.
Additionally, fish oils contain large amounts of vitamin A which
can be harmful in pregnancy, so we would not recommend this if you
are thinking of having a baby.
At present, no complementary therapies have been shown to help
with antiphospholipid syndrome.