Mary, a 31 year-old female with a long history of systemic lupus erythematosus (SLE) had multiple episodes of blood clots in her legs, as well as several miscarriages.  After her doctor ordered her to have anti-cardiolipin antibody and lupus anticoagulant blood testing, a diagnosis of anti-phospholipid syndrome complicated with SLE was made.

Anti-phospholipid syndrome (APS), an autoimmune disorder, is associated with antiphospholipid antibodies (APL) that end up attacking our own tissue.  Even though there are several types of APL antibodies, the most widely measured are the lupus anticoagulant and the anticardiolipin antibody.  These antibodies react with phospholipids, a type of fat molecule within a normal cell membrane, and then interfere with the normal coagulation process.  This disruption thus can create thrombosis (blood clotting) within the veins, placenta, or arteries, which may lead to complications such as blood clots in the legs or lungs, recurrent miscarriages, stroke, and even heart attack.

APS commonly affects people with lupus.  One-third of patients with lupus have anti-phospholipid antibodies.  However, only one-third of patients with APL develop complication as a result of these antibodies.  On occasion, APS can affect otherwise healthy individuals without lupus.

If a person has the anti-phospholipid antibody, but has never had a thrombotic complication, treatment is not currently recommended.  A low dose of aspirin, ticlopidine, or hydroxychloroquine (Plaquenil) may decrease or even prevent the frequency of thrombotic events and therefore is recommended for patients with risk factors of thrombosis.

When there is occurrence of thrombotic complication, such as a blood clot, treatment may depend on where the clot occurred (arterial or venous).  In general, treatment consists of “thinning” the blood to prevent future clots, which can usually be achieved by treating with heparin or Coumadin (warfarin).  Unfortunately, arterial blood clots that can result in heart attack or stroke require general, long term, or lifelong treatment with Coumadin.

For pregnant women with anti-phospholipid antibodies, no treatment may be necessary, as long as previous pregnancies, if any, were uneventful.  However, when a pregnant woman with APL antibody has a history of recurrent miscarriage, treatment is indicated to prevent pregnancy complications.  At this point, the most commonly used treatment includes using heparin injections with low dose aspirin.  Unfortunately, the efficacy of treatment is very unpredictable.

“Catastrophic antiphospholipid syndrome” occurs when patients experience repeated thromboembolic insults despite the prescribed treatment with aspirin, anti-malarial, and even therapeutic warfarin.  This rare condition is extremely difficult to treat and generally results in the use of plasmapheresis or intravenous gamma globulin, depending on the individual case.

In a nutshell, people who have had recurrent thrombotic events or women with a history of multiple miscarriages should be checked for antiphospholipid antibodies in order to determine if treatment is required.