Syndrome

[Guest guest]

Just something to be AWARE of: HUGHES SYNDROMEhttp://www.hughes-syndrome.org/overview.htmThere

are two major `new' diseases of the late twentieth

century –

AIDS and Syndrome ..."

Dr. Vilardell, Barcelona.

Below is an overview of Syndrome which should provide

you with valuable background information.

History

Primary

or Secondary?

Catastrophic

Antiphospholipid Syndrome

How

common is the Syndrome

Related

Diseases

History

In

1983 Dr Graham and his team in London described in detail

a condition - often known as "sticky blood", in

which there was a danger of thrombosis.

This condition – easily diagnosed by simple blood tests,

affects millions of people throughout the world. The good news

is that once diagnosed, the disease can, in most people, be

treated, and further thrombosis (clotting) prevented.

Patients with the

syndrome were at risk, both from vein thrombosis (including

DVT's), and in some, more dangerous arterial thrombosis,

including a risk of strokes and heart attacks. In women with

the syndrome, the "sticky blood" is unable to

get through the sensitive small blood vessels in the placenta

to the fetus, and there is a risk of miscarriage.

The discovery of

the condition came from careful clinical observation. Whilst

treating patients with a condition called lupus, Dr

noted that some of his lupus patients had a tendency to blood

clots, to headaches and even strokes and, in pregnancy, to

clotting of the placenta and miscarriage.

Furthermore,

Dr recognised that this group of patients could be

distinguished by a specific blood test – the detection

of so-called "antiphospholipid antibody". He immediately

recognised that the Syndrome could also occur without lupus

– indeed, in the vast majority of patients, there was

no evidence of Lupus, hence the name `primary'

antiphospholipid syndrome for these patients.

Dr gave

the syndrome the name antiphospholipid syndrome (or APS).

In the mid 1990s international colleagues re-named the syndrome

` Syndrome' to honour the doctor who described

it.

The following extract is taken from one of Dr '

original reports in 1983:

Between 1983 and 1986, Dr and his team published a

series of reports describing in great clinical detail the

spectrum of the syndrome including migraine, DVT, strokes,

movement disorder, spinal cord lesions, organ thrombosis including

brain, heart, brain, kidney, liver and lung as well as the

occasional abnormalities in blood platelet count, and of course,

the all important tendency to miscarriage.

Primary or secondary?

The antiphospholipid

syndrome was first described as a complication of the disease

`lupus'. However, Dr immediately realised

that many cases – indeed probably the vast majority did

NOT have any evidence of lupus. This gave rise

to the term `Primary Antiphospholipid Syndrome'

(PAPS).

For those patients where the clotting tendency is secondary

to another disease such as lupus, the condition is often called

`Secondary Anitphospholipid Syndrome'.

It should be stressed that the majority of patients with `Primary'

APS ( Syndrome) do NOT go on to develop

lupus in later life. The inter-relationship between lupus and

APS ( Syndrome) is highlighted in this diagram, taken

from the book ` Syndrome: A patients guide', please

see our publications if you want

to order this book.

Catastrophic Antiphospholipid Syndrome

This is the most

feared complication of Syndrome. Fortunately is it extremely

rare – but when it occurs it is an "all stops out"

medical emergency. The most commonly cited scenario is an individual

with antiphospholipid antibodies (aPL) who appears to be well

– often on no treatment – who suddenly starts to

develop widespread clots. The clots involve any or all of the

vital organs – the lungs, the liver, the adrenals, the

brain. The

triggering factor(s) for this `gear-change' is

unknown, though in a number of patients an infection such

as a virus, sore throat or chest infection seems to start

the process. Another rare cause is the stopping of anticoagulant

treatment in a known aPL patient.

How common is the Syndrome?

In

the world of obstetrics, Syndrome is now recognised as

the most common treatable cause of recurrent miscarriage.

From the world literature to date, a rough "1 in 5"

rule applies: Syndrome accounts for approximately:

1 in 5 Deep

Vein Thrombosis ('DVTs')

1 in 5 young

strokes (under 45)

1 in 5 recurrent miscarriages

Add to that the as-yet unknown number of migraine, Alzheimer's and Multiple Sclerosis sufferers who actually have Syndrome, then the prevalence figure in the population could be as high as at least 1% if not more – truly one of the `new' diseases of the late twentieth century.

Related Diseases

Lupus

This is a common `autoimmune' condition affecting

up to 1 in 1000 of the female population. The immune system

is overactive, with the resultant over-production of a large

number of antibodies. In approximately 1 in 5 lupus patients,

these antibodies include antiphospholipid antibodies, with the

resultant problems of thrombosis and miscarriage.

Sjögren's Syndrome

In many ways, this syndrome which causes dry eyes, dry mouth

and aches and pains, can be regarded as a milder version of

lupus, usually affecting a slightly older age group (50-60

year olds). Again, some people with Sjögren's Syndrome

product antiphospholipid antibodies and can develop features

of Syndrome.

Fibromyalgia

Patients with widespread aches and pains are sometimes diagnosed

as having fibromyalgia or `M.E.'. Some of these

patients, in fact, may have one of the conditions described

here such as Sjögren's Syndrome.

Thyroid disease

Abnormalities of the immune system can also affect the thyroid

and it is not uncommon, for example, to find a history of

over-active of even under-active thyroid disease in relatives

of Syndrome patients.

Other clotting disorders

A number of other clotting ("pro-thrombotic")

disorders have been described, and clearly, must be considered

when making the diagnosis.

The commonest is

factor V Leiden – a hereditary condition which leads

to vein clots but not artery clots or strokes. Others rarer

conditions include disorders of prothrombin, protein C and

protein S.