Patient Frequently Asked Questions

• Hughes Syndrome is described as an autoimmune disease, what does autoimmune mean?
  
  'Auto' means 'self', so autoimmune literally means that the immune system fights the body itself. Instead of fighting and attacking the bad tissues such as viruses, it turns on itself and attacks the good tissues. Many tissues such as thyrotoxicosis, lupus and some types of anaemia may have an ‘autoimmune’ mechanism.
  
  
• What are Antiphospholipid Antibodies?

  Antibodies are blood proteins important in defence, for example, against infection. Some antibodies appear to ‘turn against’ the body’s own tissues. Antiphospholipid antibodies appear to alter the ‘phospholipid’ skin that lines the blood vessels of organs.

  
  
• Are there different forms of Hughes Syndrome?

  Clinically – yes. Some people have few clinical problems, while in others, the thrombosis problem is severe and recurrent. In yet others, the clinical problem appears confined to recurrent miscarriage with no history of further thrombosis.

  
  
• What causes Hughes Syndrome?
  

  The exact cause is unknown, but it is likely to be a combination of factors which, in general, contribute to the likelihood of having a thrombosis. These include smoking, immobility (related for instance to the thrombosis seen after long flights) and the contraceptive pill. There is a tendency for the disease to run in families and there may be a family history of clots, of miscarriages, or of other autoimmune diseases such as lupus and thyroid problems.

  A person's genetic make-up and exposure to certain trigger factors may provide the right environment in which Hughes Syndrome can develop.
  Occasionally the thrombosis occurs during an infection such as a sore throat. However, in the some people the thrombosis comes 'out of the blue' and past medical history (e.g. migraine, recurrent miscarriages) helps to make the diagnosis of Hughes Syndrome.
  
  
• Can you catch Hughes Syndrome?

  No – it is not infectious.

  
  
• Is Hughes Syndrome a fatal disease?

  Unfortunately it can be – often before the true diagnosis is fully established – one of the causes of sudden collapse is a lung clot (pulmonary embolism) or stroke. Once the diagnosis is made, adequate anticoagulation should protect against a recurrence.

  
  
• How common is Hughes Syndrome?

  Probably very common – possibly as high as 1 in 500 of the population.

  
  
• Is Hughes Syndrome hereditary, can my children be tested?

  It is suspected that people inherit something from their parents that predisposes them to develop Hughes Syndrome. They are not necessarily pre-destined to develop Hughes Syndrome, but they may be more susceptible. Relatives of Hughes Syndrome patients have an approximately 5-12% greater tendency to get the disease if family members have it.

  
  
• If you have lupus, does that mean you also have Hughes Syndrome?

  No, 1 in 5 patients with lupus have Hughes Syndrome and an increased risk of clotting. The reverse however, does NOT apply and MOST people with Hughes Syndrome do NOT have lupus.

  
  
• What is INR?

  INR (International normalized ratio) is a blood test which refers to the thickness of the blood on Warfarin treatment. This test compares the tendency for the patient's blood to clot against a standard blood result.
  A ratio of “1” is ‘normal’
  A ratio of “2” is ‘half’ thick
  A ratio of “3” is ‘third’ thick – and so on
  Patients with Hughes Syndrome and severe clotting problems such as stroke usually need an INR of around 3.

  
  
• What lifestyle changes must be made to accommodate Hughes?

  Smoking and the oral contraceptive pill probably increases the risk as does oxygen pressure changes such as high altitude flying or diving. At the present time, is it not known if any particular diets affect the disease.

  
  
• Why is it difficult to diagnose?

  In theory, diagnosis is easy. The blood tests are simple and cheap. The doctor simply needs to think of the possibility of Hughes Syndrome.
  Having said that, the features can seem so unconnected (e.g.memory loss, previous DVT, headaches, previous miscarriage and ‘multiple sclerosis’) that the diagnosis may not immediately spring to mind.

  
  
• What are the aCL and LA tests?

  These are the two blood tests that are used to diagnose Hughes Syndrome.
  ‘aCL’ stands for anticardiolipin
  ‘LA’ stands for Lupus anticoagulant (an extremely confusing name - and not a test for lupus)

  
  
• Do all Hughes patients have the same symptoms?

  No. The diversity of symptoms is wide and can include headaches, flashing lights, giddiness, unsteady gait, cold circulation, and so on - or, just to make life more difficult - sometimes none of the above.
  The hope is that with increasing recognition of Hughes Syndrome by physicians and patients worldwide, more widespread blood testing for antiphospholipid antibodies [aCL and LA] will bring many more people to the effective treatment.

  
  
• Does taking Aspirin affect the test results?

  No. The test checks for the cause not the treatment - however patients on Warfarin cannot have the LA test.

  
  
• Can a cold or virus affect the test results?

  No.