Diagnostics and treatment of thrombotic antiphospholipid syndrome (APS): A personal perspective

Antiphospholipid Syndrome (APS) is a condition characterized by the occurrence of thromboembolic events and/or pregnancy loss combined with one laboratory criterion among Lupus Anticoagulant- LAC, anticardiolipin -aCL, and anti β2-Glycoprotein I -aβ2GPI antibodies. Several hypotheses were put forward to explain the causal role of antibodies in the clinical events but none is fully convincing. Current laboratory diagnosis is based on three tests (LAC, IgG/IgM aβ2GPI and IgG/IgM aCL antibodies). The triple-positive profile (all the three tests positive, same isotype) is associated with a higher risk for thrombosis. The mainstay of therapy in thrombotic APS is anticoagulation, with VKAs being the cornerstone. Low dose aspirin in combination or alone may have a role in arterial thrombosis, and in primary thromboprophylaxis. The Non-Vitamin K Antagonists Oral Anticoagulants (NOACs) role in the therapy of APS is under investigation but not verified. Alternative treatment options including rituximab and eculizumab have been successfully reported in few cases of catastrophic APS.