What is antiphospholipid syndrome?

Antiphospholipid syndrome, also known as Hughes Syndrome, is an autoimmune disorder which can cause blood clots anywhere in the body. It involves the production of antibodies called antiphospholipids, which attack the proteins on the outside of your blood cells.

As well as causing blood clots, antiphospholipid syndrome can result in complications during pregnancy.

Prognosis of the condition

The symptoms of antiphospholipid syndrome vary greatly from person to person. For some people, blood clots will be minor and limited to peripheral arteries, whereas blood clots in other people could result in a heart attack or stroke. Virtually any part of the body can be affected – so the condition can be extremely unpredictable.

Catastrophic antiphospholipid syndrome (CAPS) involves blood clots in multiple organs occurring over a period of a few days to a few weeks. This can often be fatal, but CAPS affects less than 1% of people with antiphospholipid syndrome.

What are the symptoms?

In addition to blood clots, antiphospholipid syndrome can cause the following symptoms:

• livedo reticularis – a blotchy red or blue rash
• chronic headaches and migraines
• extreme tiredness
• pins and needles in your arms or legs
• swelling and redness in the veins in your legs
• pregnancy problems such as frequent miscarriages or premature birth due to high blood pressure

How is it diagnosed?

Initially the doctor will carry out a medical assessment, asking about your symptoms, any past blood clots, and any unexplained miscarriages or premature births.

In most cases a diagnosis involves taking blood tests to detect antiphospholipids. You’ll need two blood tests, spaced at least 12 weeks apart, to rule out the possibility of a short-term development of antiphospholipids due to medication or infection.

What causes antiphospholipid syndrome?

The cause of antiphospholipid syndrome is unknown. Some people with antiphospholipid syndrome have a family member with the condition, but the majority do not.

Antiphospholipid syndrome occurs in 1-5% of the healthy population, but is much more common in people with autoimmune disorders such as:

• systemic lupus erythematosus
• rheumatoid arthritis
• systemic sclerosis
• giant cell arteritis

You can also get antiphospholipid antibodies if you’ve had an infection, sickle cell disease, or malignant lymphoma.

The most common group of people to be affected are women of a childbearing age.

How is it treated?

Antiphospholipid syndrome is generally treated with medication to thin the blood and reduce the chances of blood clots. In the past warfarin was the only anticoagulant drug available, but a new class of drugs known as DOACs have become available in recent years, so your doctor will discuss the benefits and drawbacks of each option. You might also be prescribed an antiplatelet medication such as aspirin.

These drugs will usually have to be taken daily for the rest of your life. During pregnancy you might additionally need to take heparin.

Finally there are some things you can do to reduce your blood pressure and therefore your chances of blood clots, such as:

• exercising regularly
• maintaining a healthy weight
• eating a healthy balanced diet
• stopping smoking

Antiphospholipid syndrome is treated by various specialists, including haematologists, rheumatologists and gynaecologists.