The anti-phospholipid antibody syndrome (Hughes syndrome): therapeutic aspects

Despite the enormous amount of work focused on the pathogenesis and clinica l manifestations of the Hughes or anti-phospholipid syndrome (APS), there i s little published on management, Usually, the diagnosis of APS is made aft er the first thrombotic event, when a thrombophilia screen is performed. Th ese patients have a high risk of recurrent thromboses and current therapy c entres on the use of thromboprophylaxis with warfarin. However, a number of clinical questions keep recurring: do arterial and venous thrombosis requi re the same intensity of anti-coagulation? When should warfarin be stopped! Should patients who develop thrombosis when other risk factors (oral contr aceptive pill, prolonged resting etc.) are present be treated like those wi thout any risk factors but the presence of anti-phospholipid antibodies (aP L)? How to manage a patient with recurrent thrombosis despite a high intens ity anti-coagulation (International normalized ratio (INR) between 3.0-4.0) ? Since many of the patients with aPL are fertile women, a substantial group of patients are diagnosed after recurrent pregnancy loss. Low-dose aspirin for those patients without previous thrombosis and aspirin plus heparin for patients with a history of thrombotic events are the current therapeutic o ptions. However, some questions remain unanswered: does the addition of hep arin to low-dose aspirin in women with first trimester recurrent miscarriag e but without previous thrombosis improve foetal outcome over and above asp irin alone! Which is the best therapeutic regime during pregnancy for patie nts with aPL-associated stroke! When should high-dose intravenous gammaglob ulin be considered! Finally, very little is known about the risk of thrombosis in individuals p ositive for aPL but still free of thrombosis. Should these individuals rece ive any treatment! If so, which one! In this review we attempt to address some of these questions taking into ac count available data from retrospective and prospective studies and our own clinical experience.