The antiphospholipid (Hughes) syndrome Latest advances in pathogenesis and treatment

Haema 2014; 5(1):24-34

by Paraskevi Kotsi

Blood Center and Center for Bleeding Disorders, Laiko General Hospital, Athens, Greece

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The antiphospholipid (Hughes) syndrome (APS), is characterized by autoantibody production directed against phospholipids leading to an increased risk of arterial and venous thrombosis, recurrent pregnancy loss or other obstetric features. It can occur as a primary syndrome or in association with other disorders mainly with SLE (systemic lupus erythematosus). The antibodies can be detected by ELISA or coagulation tests. The latest advances in pathogenesis implicate oxidative stress and B-cell responses leading to new therapeutic approaches, including more focused utilization of existing therapies and the introduction of biological therapies. B cells have a critical role in these disorders and B-cell depletion or suppression improves disease in some patients. Studies also suggest a role for the innate immune system in disease pathogenesis, suggesting further future targets for biological therapies over the next few years. The consensus is once a patient has had a thrombosis, would be anticoagulated for life due to the high risk of further thrombosis. There is no sufficient evidence in most ‘straight- forward’ cases of APS that immunosuppression have any beneficial effect. The potential exception to this is in rare patients (about 1% of APS cases) with the catastrophic APS. Some recent papers have described the use of rituximab with beneficial effect in resistant cases. Statins and many other drugs also have antithrombotic and immunomodulatory effects could be used as adjunctive therapies but untill now no studies are available.