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“It is not necessary to have ALL symptoms
to be diagnosed with
Hughes Syndrome (antiphospholipid syndrome - APS)”
Hughes Syndrome (APS) is sometimes called 'sticky blood syndrome'.
This is because people with it have an increased tendency to
form clots in blood vessels (also known as thromboses). Any
blood vessel can be affected including the veins and the arteries.
Click here for a
comprehensive snapshot.
The main symptoms of Hughes Syndrome can include any of the
following:
Headache or migraine
Did you suffer from headaches or migraine as a teenager? So
often this is one of the major features of the illness. Sometimes
the headaches disappear in the 20’s to return with a vengeance
in the 30’s or 40’s. This is a most important feature
of Hughes Syndrome, and a symptom which sometimes improves dramatically
when treatment is started. Often migraine
features such as flashing lights and zigzag patterns accompany
the headaches found in Hughes Syndrome patients.
Giddiness
For reasons not completely
understood, the brain appears particularly sensitive to the
clotting effects of antiphospholipid antibodies, and one of
the ways in which it reacts to "sticky blood" affecting
its oxygen supply is to cause balance disorders. Many patients
complain of feeling giddy or "slightly drunk" and
this can naturally lead to accidents.
Memory loss
 When
the brain is starved of oxygen (blood supply) it only has a
limited number of ways of complaining, and a common symptom
of Hughes Syndrome is memory loss. Many patients feel that they
are developing Alzheimer’s disease when they can’t
remember names of friends and family, forget their shopping
lists and get their words and sentences muddled. One of the
most dramatic observations in the whole of medicine is the improvement
of memory (and the disappearance of the headaches
and ‘fog’) which patients observe when blood thinning
medicine is started.
Visual disturbance
In
addition to the flashing lights and zigzag patterns which can
accompany headaches and migraines, the person with Hughes Syndrome
can experience double vision or sudden visual loss. This can
be caused by the brain reacting to disturbances in its supply
of blood or by the veins and arteries in the eye being affected.
Skin disorders
 Many
Hughes Syndrome patients complain of ‘cold circulation’
and this sometimes manifest as a blotchy appearance of the skin
of the arms and legs, described in medical textbooks as “livedo
reticularis” or, more prosaically, as “corned beef
skin”. It can also cause repeated sores (ulcers) and bumps
(nodules) of the skin.
Thrombosis - DVTs
Thrombosis, or blood
clots, can occur in the untreated patient at any time and in
any part or organ of the body. For example a deep vein thrombosis
or DVT can form in the arm or leg after a long journey, or in
some women, after starting the contraceptive pill. Clots in
the veins can cause inflammation of the veins (thrombophlebitis)
of the legs with pain in the thigh or calf, swelling of the
leg, and sometimes a visible red, thickenening blood vessel.
Thrombosis can also affect vital organs such as the eye, liver
and kidney.
Heart attack
Hughes Syndrome
can lead to heart attacks and heart valve problems that can
mimic bacterial endocarditis, and can create clots in the upper
chambers of the heart. Up to 20% of young people (under 45)
who have a heart attack have antiphospholipid antibodies.
Stroke
 The
most feared complication of blood clotting is strokes –
paralysis. There are many causes of strokes – for instance,
raised blood pressure, but most surveys show that 1 in 5 “young”
strokes (under the age of 45) are now associated with Hughes
Syndrome (APS). In other words, in an ideal world, up to 1 in
5 “young” strokes could be prevented. This observation
is very important. Even now, in the age of easy blood diagnoses
of Hughes Syndrome, many patients are not receiving adequate
anti-coagulant treatment of their “sticky blood”,
and are suffering early mini-strokes or TIAs (transient ischemic
attacks) or more permanent strokes.
Pulmonary embolism
A lung embolus (pulmonary
embolism) occurs when a blood vessel supplying the lung becomes
clogged up by a clot - a lump of coagulated blood. Blood clots
in the lung can cause chest pain, shortness of breath and rapid
breathing. Repeated clots can cause elevated pressure in the
blood vessels around the lungs (pulmonary hypertension), which
may cause the person to be constantly short of breath. Larger
emboli in the lungs can be lethal.
Multiple sclerosis-like features
Some people
with Hughes Syndrome develop a syndrome which is very similar
to multiple sclerosis where they have numbness or pins and
needles, double vision or loss of part of the field of vision,
and have difficulty walking. Consequently, one of the main
alternative diagnoses in patients with Hughes syndrome is
multiple sclerosis, and the clinical team here are continually
treating patients who had been previously misdiagnosed with
“multiple sclerosis”.
Gastrointestinal disorders
Hughes Syndrome can
affect the blood supply to the intestines, causing abdominal
pain, fever and blood in the stool. Antiphospholipid antibodies
can also cause a condition called Budd-Chiari syndrome, in which
a blood clot prevents blood from flowing out of the liver and
the person may then experience nausea, vomiting, jaundice (yellow
skin), dark urine and the swelling of the abdomen.
Pregnancy problems
One miscarriage is
a disaster. Two is worse. Imagine the suffering of women who
have 3, 5, 7 or even 12 pregnancy losses, and sometimes as late
as the last few weeks of their pregnancy.
We now know that Hughes Syndrome is the most common treatable
cause of recurrent miscarriage: depending on which study you
quote, the figure is up to 1 in 5. Furthermore, late pregnancy
loss, fortunately an unusual problem in pregnancy, is very strongly
associated with Hughes Syndrome as is pre-eclampsia, placental
abruption and intra-uterine growth restriction.
For the sake of a simple blood test, patients with miscarriage
or late pregnancy loss can be tested for Hughes Syndrome. Treatment
of these patients has proved one of the true successes of modern
medicine, the successful pregnancy rate rising from a previous
low of fewer than 20% to figures now in the region of 75-80%
success rate. Treatment is with low-dose aspirin and/or heparin.
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