Hughes syndrome

6 February, 2009

Hughes syndrome is a condition where clots (thromboses) form in the blood vessels. It is sometimes called 'sticky blood', and is also known as antiphospholipid syndrome (APS). The condition is named after Dr Graham Hughes, who discovered it in 1983.
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Overview

Introduction

Hughes syndrome is a relatively common cause of recurrent stroke, heart attack, thrombosis and miscarriage. It affects both men and women, but is mainly seen in women.

There are two types of Hughes syndrome:

primary, where Hughes syndrome develops by itself and is not caused by another condition, and
secondary, when another condition causes you to develop Hughes syndrome. These conditions are typically autoimmune disorders, such as lupus (a condition where the body develops antibodies that attack healthy tissue).

Symptoms

Symptoms of Hughes syndrome

The symptoms of Hughes syndrome will depend on where clots in your blood occur, and where they move to. A clot in your blood may cause the conditions outlined below.

Deep vein thrombosis - a blood clot in your leg. This results in a painful swelling in your leg, normally starting in your calf.
Pulmonary embolism - a blood clot that moves into your lung. This may cause you to have a shortness of breath, chest pain, or it may result in you coughing up a small amount of blood.
Repeated miscarriages or still births - this can happen when blood clots block the supply of blood to the foetus. If you have had three, or more, consecutive miscarriages, you should be tested for Hughes syndrome.
Heart attacks or stokes - this can happen when blood clots block the supply of blood to your heart or brain. As you get older, your blood vessels can get narrower (atherosclerosis), leading to a higher chance of a blood clot. However, if you have Hughes syndrome, this can happen at a much earlier age. Therefore, if you are under 45 years of age, and you have had a stroke or a heart attack, you should be tested for Hughes syndrome.

Other less common symptoms Hughes syndrome include:

headaches or migraines,
rashes - a 'net-like' rash may appear on your wrist or your knee which is sometimes known as 'livedo reticularis',
bleeding from nose or gums,
sudden uncontrollable movement of your body, and
memory loss.

Causes

Causes of Hughes syndrome

The exact trigger of Hughes syndrome is unknown, but research has shown that antiphospholipid antibodies play a role in causing blood clots.

Antibodies are produced by your body's immune system to attack harmful bacteria, or viruses. Hughes syndrome causes your body to produce antibodies that mistakenly attack a type of fat found in your blood called phospholipids. This can lead to clotting of your blood.

Diagnosis

Diagnosing Hughes syndrome

Hughes syndrome is usually diagnosed using a combination of different methods.

Your GP will take a look at your medical history and will ask you if you have had a history of clotting problems, heart attacks or strokes (particularly at a young age), migraines, or memory loss.

Women will be asked about previous pregnancies, and whether there were any complications, or a history of miscarriages.

You may have a physical examination to check for a rash, and your blood pressure, cholesterol and glucose levels may be tested to rule out other causes of blood clots, such as hypertension and diabetes.

If the diagnosis uncovers evidence that suggests that you might have Hughes syndrome, your blood will be tested for antiphospholipid antibodies. The same test is usually run 6-8 weeks later to confirm the diagnosis.

If the tests show the presence of antiphospholipid antibodies, you will be diagnosed with Hughes syndrome and your treatment will begin.

Treatment

Treating Hughes syndrome

Once Hughes syndrome has been diagnosed, the treatment is relatively straightforward, and if you are being treated for the condition, you are much less likely to experience any further blood clots.

Aspirin

The main aim of treatment for Hughes syndrome is to thin the blood. The vast majority of people find that one-quarter of an aspirin (which has blood thinning properties) per day is all that is required to thin the blood.

Warfarin and heparin

In cases where aspirin is not suitable, or a blood clot is present, blood thinning medication called warfarin is used. This can be taken in pill form. There is a small chance that warfarin can cause birth defects, so it is not recommended during pregnancy. In such cases, heparin is recommended. This is a similar medication to warfarin and is considered safe to take during pregnancy. However, unlike warfarin, heparin has to be injected.

For Hughes syndrome, long term treatment is usually required. If you develop the condition, you may need treatment for the rest of your life because suddenly stopping could increase the risk of blood clots re-occurring.

Prevention

Preventing Hughes syndrome

As little is known about what causes Hughes syndrome, there is not much you can do to prevent the condition developing.

However, if you have been diagnosed with Hughes syndrome, there are steps you can take to reduce the risks of blood clots occurring. For example, you should: