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Hughes Syndrome Treatments

  • General treatments
  • Other drugs
  • What can I do to help myself?
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    General treatments

    Due to the increased tendency to clot, the main aim of treatment is to 'thin' the blood, so that this tendency is reduced. This is usually achieved for most patients by providing anticoagulant (literally anti-clotting) therapy based on the extent of the clotting.

    There are currently three main medications - aspirin, heparin or warfarin. For many thousands of patients, one "junior" aspirin a day (approximately 75-80 mg, or one quarter of a regular aspirin) is all that is required. Its main action is to make the platelets of the blood less "sticky" and is sufficient to hold off clotting. It is the main medication used in pregnancy in Hughes Syndrome patients with previous miscarriage.

    For those who have actually had a thrombosis, the choice is between heparin and warfarin. Heparin is a very useful anticoagulant but unfortunately is only available as an injection, and cannot be given long term because of an increased risk of osteoporosis. It is, however, used in some Hughes Syndrome pregnancies, as warfarin is potentially toxic to the developing foetus.

    For most people with thrombosis, warfarin is the treatment of choice. Used for many decades warfarin is the 'gold standard' anticoagulant tablet and provided that the anticoagulation dose is monitored carefully to test that the patients blood is thinning to the correct degree, it is an extremely safe drug. Those taking warfarin are monitored with a standard clotting test called the INR - a procedure carried out either in GP's or hospital anticoagulant clinics, or, increasingly, by the patient themselves with self-test machine.

    For other symptoms, most patients are treated on a case-by-case basis, or ailment-by-ailment basis. Plaquenil is an effective treatment option for general symptoms such as rash, fatigue and pain.

    Some notes on the three treatments are given here:

    Aspirin 

    • Low dose aspirin (‘junior aspirin’, ‘baby aspirin’) – at a dose of between 75mg to 100mg a day is known to be an effective anti-clotting agent, making the blood platelets less sticky.
    • Safe. Side effects are rare (indigestion; allergy especially in asthmatics)
    • Not sufficient treatment of those with major thrombosis or stroke

     Heparin 

    • Newer “low molecular weight” heparin (such as ‘Clexane’ or ‘Fragmin’) has replaced older preparations
    • Only available as injection
    • Useful as a ‘first line’ (immediate action) treatment in thrombosis, prior to warfarin use
    • Safe in pregnancy

    Warfarin (Coumadin) as a Lupus Treatment 

    • Despite being dismissed as ‘rat poison’ by some journalists, warfarin is an extremely safe and effective medicine, it has a good long track record having been around since 1950s
    • Side effects are rare – the only vital issue is to keep the dosage correct
    • The thinning of the blood achieved by warfarin is measured on an ‘International’ scale called ‘INR’ ‘Normal’ is “1” – half thick blood is “2” and one third blood is “3” (think of it as ‘full cream’, half cream’, ‘skimmed milk’).
    • Not allowed in preganancy

    Other Drugs as a Hughes Syndrome Treatment

    Clopidogrel (PLAVIX) as a Lupus Treatments

    This drug has a mechanism similar to aspirin in making platelets less ‘sticky’. Although expensive, there are real reasons for looking at Plavix. Firstly, Plavix does not have the irritant effects on the stomach which can occur with aspirin (fortunately rare with the low dose). Neither is it a problem in asthmatics, who may prove allergic to aspirin. Thirdly, huge studies in cardiology, where aspirin and plavix have been compared in the prevention of heart attacks, have thrown up some evidence that, under certain circumstances, plavix may have an edge over aspirin.

    In Hughes syndrome, where clotting and platelets are key, similar studies comparing the two agents will be warmly welcomed.

    Intravenous Immunoglobulin (IVIG) as a Lupus Treatments

    This consists of an intravenous preparation of protein (globulin) pooled from a large number of donors. It is extremely expensive, and its action is short-lived. Some years ago, it was found to help in some cases of thrombocytopenia (low platelets) and has since been used in a variety of autoimmune diseases with mixed success. Despite, its apparent limitations, it has a good safety record, and is being tried in some patients with Hughes’ Syndrome, particularly those with low platelet counts.

    Immunosuppressives as a Lupus Treatment

    These drugs (most commonly azathioprine and methotrexate) are widely used in auto-immune diseases such as Lupus and rheumatoid arthritis. They have proved disappointing in patients with primary APS. So also has plasma exchange – an attractive idea for removing antibodies, but with little convincing published success – as yet.

    Antimalarials as a Lupus Treatments

    Hydroxychloroquine (Plaquenil) is an extremely useful drug in Lupus and Sjögrens Syndrome. It is particularly effective in helping skin rashes, fatigue, and aches and pains. One of the additional actions of Plaquenil is as a (mild) anti-clotting agent – rather like junior aspirin. Thus, in Lupus patients with aPL it might well have extra, hidden, benefits.

    What can I do to help myself?

    Smoking is an added risk for clotting, as is the oral contraceptive pill. In theory, increasing the amount of essential fatty acids (EFAs) in your diet, particularly omega-3 EFAs found in oily fish, should help reduce the risk of thrombosis, but there are no clinical trials to suggest this is the case.

    It is worth noting that fish oils also contain large amounts of vitamin A, which may be harmful in pregnancy.

    No other 'alternative' treatments have been shown to help.

    You should be aware of the signs and symptoms of Hughes' Syndrome and report any of these to your doctor as soon as they occur.