Abstract
Persistent serum positivity for antiphospholipid antibodies (aPL) is required to diagnose
antiphospholipid syndrome (APS), an autoimmune disease characterized by recurrent
vascular thrombosis and/or pregnancy morbidity. The current therapeutic management
of patients with thrombotic APS aims at preventing recurrences and long-term complications
by attenuating the procoagulant state. There is overall consensus to reserve moderate-intensity
anticoagulation to aPL-positive patients with a previous venous thrombosis; the therapeutic
options for those with a history of arterial event comprise antiplatelet agents and
high-intensity anticoagulation. Unfortunately, thrombotic occurrences might occur
despite adequate anticoagulation, carrying a significant burden of morbidity and mortality.
The management of refractory thrombotic APS and catastrophic APS is still not clear,
warranting the issue of recommendations. Vitamin-K antagonists are limited by significant
side effects, and a careful weighting of risks and benefits should be performed to
reserve the optimal treatment to each patient. To overcome these limitations, novel
oral anticoagulants have been introduced in the market, but their efficacy in thrombotic
APS has still to be unraveled. The poor safety profile and the scarce efficacy of
drugs acting on the coagulation cascade explain why novel therapeutic approaches are
currently under investigation, to identify pharmacological tools specifically counteracting
aPL-mediated prothrombotic effects.
Keywords
antiphospholipid antibodies - antiphospholipid syndrome - thrombosis - treatment
