Antiphospholipid syndrome

Text A
Antiphospholipid syndrome(APS), sometimes known as
Hughes syndrome, is a disorder of the immune system that
causes an increased risk of blood clots.
An accurate diagnosis of Antiphospholipid syndrome (APS)

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is important because blood clots can have serious
consequences.

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A diagnosis of APS can usually be confirmed if you have
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had:
• 1 or more confirmed blood clots
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• 1 or more unexplained late miscarriages at or after

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week 10 of your pregnancy

• 1 or more premature births at or before week 34 of your
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pregnancy
• 3 or more unexplained early miscarriages before week
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10 of your pregnancy
It’s not clear why these abnormal antibodies are produced,
or why many people have antiphospholipid antibodies but
don’t develop blood clots. A combination of genetic and
environmental factors is thought to be responsible.
Antiphospholipid antibodies
Antibodies are proteins produced by the immune system to
help fight off infection and illness. In APS, the immune

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system produces abnormal antibodies that rather than
attacking bacteria and viruses, mistakenly attack proteins
found on the outside of cells in the blood and blood vessels.
It’s not known how this causes the blood to clot more easily.
Genetic factors
Research into the genetics around APS is still at an early
stage, but it seems the genes you inherit from your parents
may play a role in the development of abnormal

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antiphospholipid antibodies.
APS isn’t passed down directly from parents to children in

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the same way as other conditions, such as haemophilia and
sickle cell anaemia.
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Studies have shown that some people with APS have a faulty
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gene that plays a role in other autoimmune conditions, such

as lupus.
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Environmental factors
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It’s thought that one or more environmental triggers may

be needed to trigger APS in some people.
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Environmental factors that may be responsible include:

• Viral infections, such as the cytomegalovirus (CMV) or
parvovirus B19
• Bacterial infections, such as E. coli (a bacteria often
associated with food poisoning) or leptospirosis (an
infection usually spread by certain animals)
• Certain medications, such as anti-epileptic medicine or
the oral contraceptive pill

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Another theory is that many people with abnormal
antiphospholipid antibodies only go on to develop APS if
they have a higher risk of developing blood clots.
For example, if they:
• Eat an unhealthy diet, leading to high cholesterol levels
in the blood
• Don’t do enough exercise
• Take the contraceptive pill or hormone replacement

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therapy (HRT)
• Smoke

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• Are obese
Text B
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In antiphospholipid syndrome (APS), the immune system
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produces abnormal antibodies that make the blood

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“stickier” than normal. This means people with APS are

more likely to develop blood clots in their veins and arteries,
ET

which can cause serious or life-threatening health

problems.
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These include:
• High blood pressure
• Deep vein thrombosis (DVT)
• A stroke or a transient ischaemic attack (TIA) (“mini-
stroke”)
• Heart attacks
• Pulmonary embolism (a blockage in one of the blood
vessels in the lungs)

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People with APS may also experience any of the following
symptoms:
• Balance and mobility problems
• Vision problems, such as double vision
• Speech and memory problems
• A tingling sensation or pins and needles in your arms
or legs
• Fatigue (extreme tiredness)

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• Repeated headaches or migraines
• Pregnancy problems-Women with APS have a much

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higher risk of developing complications during
pregnancy, particularly if it’s not treated.
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Text C
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Treatment for antiphospholipid syndrome (APS) aims to

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reduce your risk of developing more blood clots.

Medication
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As part of the treatment, patient will be prescribed

anticoagulant medicine such as warfarin, or an antiplatelet
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medication such as low-dose aspirin. These work by

interrupting the process of blood clot formation. This
means blood clots are less likely to form when they’re not
needed.
Most people with APS need to take anticoagulant or
antiplatelet medication daily for the rest of their life. If can’t
take aspirin, patient may be prescribed an alternative
antiplatelet tablet called clopidogrel. Warfarin tablets are

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usually recommended if you have APS and a history of
blood clots, such as previously having deep vein thrombosis
(DVT) or a stroke. But this needs to be changed if you
become pregnant or are planning a pregnancy. If you
develop a blood clot or your symptoms suddenly become
severe, injections of an anticoagulant called heparin may be
needed.
Treatment during pregnancy

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Women diagnosed with APS are strongly advised to plan for
any future pregnancy. This is because treatment to improve

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the outcome of a pregnancy is most effective when it begins
as soon as possible after an attempt to conceive. Some
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medications used to treat APS can also harm an unborn
baby.
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Treatment during pregnancy involves taking daily doses of

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aspirin or heparin, or a combination of both. This depends

on whether patient have a history of blood clots and
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previous complications during pregnancy. Warfarin isn’t

recommended during pregnancy because it carries a small
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risk of causing birth defects. Treatment with aspirin or

heparin, or both, is usually started at the beginning of the
pregnancy and may continue for 1 to 6 weeks after you have
given birth.
Lifestyle changes
If you’re diagnosed with APS, it’s important to take all
possible steps to reduce your risk of developing blood clots.
Effective ways of achieving this include:

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 • Not smoking
• Eating a healthy, balanced diet – low in fat and sugar
and containing plenty of fruit and vegetables
• Taking regular exercise
• Maintaining a healthy weight and losing weight if
you’re obese (have a body mass index of 30 or more)
Catastrophic antiphospholipid syndrome
Catastrophic antiphospholipid syndrome (CAPS) is a rare

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but serious complication of antiphospholipid syndrome
(APS). It occurs in less than 1% of people with APS. In

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people who develop CAPS, blood clots suddenly form
throughout the body, resulting in multiple organ failure. It’s
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not clear what causes this, but 1 case in 5 occurs after an
infection, trauma or surgery. The initial symptoms can be
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wide-ranging, depending on which organs are involved.

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Symptoms may include:

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• Loss of blood supply to the tips of limbs causing them

to go dark blue or black
• Swollen ankles, feet or hands
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• Increasing breathlessness
• Tummy (abdominal) pain
• Blood in the urine
• Fits (seizures)
• Coma
The symptoms usually develop suddenly and rapidly get
worse. Immediate admission to an intensive care unit (ICU)
is required for people with CAPS so the body’s functions can

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be supported. High-dose anticoagulants are used to stop the
blood clots getting bigger while they’re slowly absorbed by
the body. But even with the best available treatment, an
estimated 50% of people die as a result of the condition.
Text D
Possible complications include:
• Recurrent (3 or more) early miscarriages, usually

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during the first 10 weeks of pregnancy
• 1 or more later miscarriages, usually after week 10 of

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pregnancy
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• Premature birth, usually at or before week 34 of
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pregnancy, which may be caused by pre-eclampsia
(where a woman develops high blood pressure during
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pregnancy)
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Livedo reticularis
Livedo reticularis is a skin condition caused by small blood
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clots that develop inside the blood vessels of the skin. It

causes the skin to take on a blotchy red or blue appearance.
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Some people also develop ulcers (sores) and nodules

(bumps).These symptoms are often more severe in cold
weather.
Superficial thrombophlebitis
Superficial thrombophlebitis is inflammation of the veins
just under your skin, usually in your leg. The symptoms are
similar to DVT, but aren’t usually as severe. The symptoms
of superficial thrombophlebitis include:

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• Swelling
• Redness and tenderness along the affected vein
• A high temperature of 38C (100.4F) or above (although
this is less common)
• The symptoms usually resolve within 2 to 6 weeks.

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Time: 15 minutes
• Look at the four texts, A-D, in the separate Text Booklet.
• For each question,1-20,look through the texts, A-D, to
find in this Question Paper
• Answer all the questions within the 15 Minute time limit
• Your answers should be correctly spelt.

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Antiphospholipid syndrome :Questions

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Questions 1-7 ee
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For each question, 1-7, decide which text (A,B,C or D) the
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Information comes from. You may use any letter more

than once. In which text can you can find information
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about.
1. Mini-stroke
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2. A diagnosis of APS
3. Immediate admission to an ICU
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4. Repeated headaches or migraines

5. Occurs in less than 1% of people with APS
6. Redness and tenderness along the affected vein
7. Cause serious or life-threatening health problems

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Questions 8- 14
Answer the questions,8-14,with a word or short
phrase from one of the texts. Each answer may
include words, numbers or both.

8. Which cutaneous condition is characterised with

mottled discolouration of the skin-----------------------?

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9. Which is an life – threatening complication of

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antiphospholipid syndrome - - - - - - - - - - - - - - - - -?
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10. What is CAPS - - - - - - - - - - - - - - - - - - - - - - - -?
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11. Which drug can be prescribed to treat APS instead

of Aspirin - - - - - - - - - - - - - - - - - - - - -?
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12. Which is the drug of choice, If symptoms of APS

suddenly become worse - - - - - - - - - - - - - - - - - - - -?
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13. Which are the bacterial infections that causes APS

- - - - -?

14. Which is an example for autoimmune disease - - -

- - - - -?

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Questions 15-20
Complete each of the sentences, 15-20, with a word
or short phrase from one of the texts. Each answer
may include words, numbers or both.
15. ----------------is a condition where a woman
develops high blood pressure during pregnancy?

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16. A combination of - - - - - - - - - - - - - - and - - - - - -
- - - - - - - may have a role to develop APS?

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17. ee
------ - - - - - ------ isn’t recommended during
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pregnancy as it may cause congenital defects?
ith
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18. Treatment of APS during pregnancy includes

taking daily doses of - - - - - - - - - - - - - - - - - - - or - - -
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- - - - - - - - - or a - - - - - - - - - - - - - - - -?
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19. In APS the immune system produces - - - - - - - - -

- - - - that make the blood - - - - - - - - - - - than normal?

20. ---------------------or - - - - - - - - - - - - - medications

may cause APS?
End of Part A
Proceed to Part B & C

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