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"What is the antiphospholipid syndrome (Hughes&amp;#039; syndrome)?  The antiphospholipid syndrome (APS) is sometimes called &amp;#039;sticky blood syndrome&amp;#039;. 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, which are thin walled and take blood towards the heart, and the arteries, which have thick muscular walls, and take blood away from the heart. As a result of this clotting tendency these patients may develop repeated clots, eg in the veins of the leg (deep vein thrombosis), or in the arteries supplying the brain, causing a stroke.  In pregnancy, the placenta can be affected by small clots, and there is an increased risk of miscarriage, particularly in mid-pregnancy.  What is the cause of the antiphospholipid syndrome?  The cause is thought to be &amp;#039;antiphospholipid antibodies&amp;#039;. Antibodies are produced by the body&amp;#039;s immune system to fight infection from bacteria and viruses. In some diseases, the immune system produces antibodies that mistakenly attack the body&amp;#039;s own tissues. The result is an autoimmune disease, eg antiphospholipid syndrome.  Phospholipids are phosphorus-containing fat molecules found throughout the body, particularly in the surrounding coat of cells - the cell membranes. The &amp;#039;antiphospholipid antibodies&amp;#039; target the body&amp;#039;s own phospholipids, and also proteins that bind to phospholipids.  What types of antiphospholipid syndrome exist?  When only the symptoms of the antiphospholipid syndrome occur, this is known as primary antiphospholipid syndrome. The secondary form occurs in association with another autoimmune disease, usually systemic lupus erythematosus. Both types are more common in women.  What are the symptoms of antiphospholipid syndrome?  The symptoms relate to abnormal clotting. Most commonly clots can develop in the veins of the legs (deep vein thrombosis), resulting in a swollen, painful leg, with the swelling usually starting in the calf. These clots in the leg may happen once, or several times.  Clots may also occur in the lung (pulmonary embolus), sometimes following a clot in the leg, which breaks off and travels in the circulation until it lodges in part of the blood supply to the lung. A clot in the lung may lead to shortness of breath, chest pain made worse by breathing in, or coughing up a small amount of blood. Some clots occur in patients soon after starting on the oral contraceptive pill (oestrogen-containing types).  Veins in other parts of the body may clot, including the eye, the kidney, the liver, and the adrenal gland.  Depending on where it occurs, clotting within an artery can result in a stroke, heart attack, or blockage of the blood supply to a limb, possibly leading to gangrene. Such arterial diseases occur commonly from atherosclerosis in old age, but in the antiphospholipid syndrome they can happen at a much younger age. Hence a patient under 50 who has a stroke or a heart attack should be tested for the antiphospholipid syndrome.  Women with antiphospholipid syndrome are at increased risk of miscarriage. Clots may form in the placenta leading to an inadequate blood supply to the foetus.  Miscarriages in antiphospholipid syndrome usually occur in the second or third trimester (week 12 or later), but can sometimes occur in the first trimester.  Fifteen per cent of women with three or more consecutive miscarriages have positive antiphospholipid syndrome tests. There are patients with antiphospholipid syndrome who have successful pregnancies without treatment, though there is a higher risk of complications towards the end of pregnancy, including pre-eclampsia, intrauterine growth retardation (slowing down of the baby&amp;#039;s growth in the uterus), and premature delivery.  Patients with antiphospholipid syndrome report a higher incidence of headaches, including migraines. A proportion of patients have memory loss, or more subtle abnormalities. Epilepsy is commoner in antiphospholipid syndrome. There is a higher incidence of chorea, also known as St.Vitus&amp;#039; Dance (abnormal sudden jerky movements).  Patients with secondary antiphospholipid syndrome may report symptoms of the underlying disease. For example, patients with underlying systemic lupus erythematosus may report features including a rash on the cheeks, skin reaction to sunlight, joint pains, mouth ulcers, excessive hair loss, dry eyes, cold and bluish fingers, and sometimes sharp chest pains.  How is antiphospholipid syndrome diagnosed?  The diagnosis depends on the history, the examination, and special tests. The doctor will ask about clotting problems in the past (any history of clots in the leg or lung), previous strokes or heart attacks (and whether they occurred at young ages), less specific clues such as headaches, migraine, memory loss, and confusion.  Women will be questioned about past pregnancies, and whether there were any complications. They will be asked specifically about any miscarriages, and at what stage of pregnancy they occurred.  A lacy, net-like, red rash known as &amp;#039;livedo reticularis&amp;#039;, is often found in antiphospholipid syndrome, particularly over the wrists and knees.  A simple blood test can detect the &amp;#039;antiphospholipid antibodies&amp;#039;. Other blood tests can check for underlying conditions, such as systemic lupus erythematosus.  What can your family doctor do?  Your GP can ask about the features that have been described above, particularly clotting problems and miscarriages and can look for the skin rash, livedo reticularis.  If your GP suspects the diagnosis, they can either send you for a blood test to look for the &amp;#039;antiphospholipid antibodies&amp;#039;, or refer you to a specialist.  When to refer to a specialist?  If the GP suspects the diagnosis from the features described by the patient, or if the specific blood test is positive, the patient will be referred to a specialist.  The specialist can be either a rheumatologist (joints and soft tissues expert), or a haematologist (blood disease expert). Both these specialists have experience in diagnosis and treatment of the antiphospholipid syndrome. They may perform further tests to confirm the diagnosis. They may also perform tests to look for damage caused by antiphospholipid syndrome, which could include other blood tests, echocardiogram (ultrasound scan of the heart), and MRI scans.  Treatment of antiphospholipid syndrome  Because of the increased tendency to clot, the main aim of treatment is to &amp;#039;thin&amp;#039; the blood, so that this tendency is reduced. This is usually achieved with a tablet called warfarin (eg Marevan), which is an anticoagulant (literally anti-clotting). This is taken daily, and regular blood tests are required to ensure that the warfarin is thinning the blood to the required degree. This blood test is referred to as the INR (International Normalised Ratio). This test compares the tendency for the patient&amp;#039;s blood to clot against a standard blood result. Warfarin treatment is given to patients who have antiphospholipid syndrome characterised by recurrent clots.  Patients with antiphospholipid syndrome who have recurrent miscarriages can also be treated, but not with warfarin as it has the potential to cause foetal abnormality if given during pregnancy. Treatment of such patients is with either aspirin (eg Caprin 75mg), or another type of anticoagulant, heparin. Sometimes both are used.  Heparin needs to be given daily during pregnancy by injection under the skin. Patients are usually taught how to do this, and most manage without difficulty. It can also be given by nurses. Unlike warfarin, there are no blood tests usually required to monitor heparin treatment.  At present the recommended treatment is low dose aspirin, providing there are no contraindications.  Some patients have positive antiphospholipid antibody tests, but have never had a thrombosis or a miscarriage. Research into the best treatment for such patients is currently being carried out.  At present, the recommended treatment is low dose aspirin. Aspirin acts on platelets (small blood cells involved in forming clots) to reduce their stickiness, and hence reduces the ability of the blood to clot.  Living with antiphospholipid syndrome  If correctly treated, the patient&amp;#039;s outlook is good. The risk of further clots both in arteries and veins is reduced in patients who receive treatment with warfarin. But the treatment needs to be long term or even lifelong, as there is a high risk of further clots in patients who stop warfarin treatment.  Patients should also reduce their risk of thrombosis from causes other than the antiphospholipid syndrome. They should stop smoking, make sure they are a healthy weight, and take regular exercise. The doctor will assess their other risk factors for thrombosis - by measuring their blood pressure to rule out hypertension, checking their glucose levels to rule out diabetes and measuring cholesterol levels.  Women who have suffered several miscarriages have subsequently had successful pregnancies. Treatments include aspirin, heparin, careful supervision by obstetricians and physicians, and regular scanning to check their baby&amp;#039;s wellbeing.  Further information on antiphospholipid syndrome (Hughes&amp;#039; syndrome)  Website: www.hughes-syndrome.org. Helpline: 020 7188 8217   A patient booklet can be obtained from Lupus UK, 1 Eastern Road, Romford, Essex, RM1 3NH. Telephone: 01708 731251.   References Khamashta MA. Management of the antiphospholipid syndrome. CPD rheumatology 1999 Vol 1 No.1.  Hughes GRV. The antiphospholipid syndrome, A historical view. Lupus 1998; 7 Suppl 2: S1-S4. Dr.Graham Hughes. Hughes&amp;#039; syndrome. A patient&amp;#039;s guide to the antiphospholipid syndrome.   Last updated 31.07.2005 "^^<http://www.w3.org/2001/XMLSchema#string>
<http://www.netdoctor.co.uk/conditions/heart-and-blood/a1174/the-antiphospholipid-syndrome-hughes-syndrome/>