How is thrombosis in antiphospholipid syndrome (APS) treated?

Updated: Sep 30, 2018
  • Author: Suneel Movva, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

Perform full anticoagulation with intravenous or subcutaneous heparin followed by warfarin therapy. Based on the most recent evidence, a reasonable target for the international normalized ratio (INR) is 2.0-3.0 for venous thrombosis and 3.0 for arterial thrombosis. Patients with recurrent thrombotic events may require an INR of 3.0-4.0. For severe or refractory cases, a combination of warfarin and aspirin may be used. Treatment for significant thrombotic events in patients with APS is generally lifelong.

No data exist regarding new oral anticoagulants (ie, direct thrombin inhibitors and factor Xa inhibitors) in APS patients. Currently, these agents can be considered in patients who are warfarin intolerant/allergic or have poor anticoagulant control. [8, 19] Two studies of the factor Xa inhibitor rivaroxaban are currently in progress: Rivaroxaban for Antiphospholipid Syndrome (RAPS) and Rivaroxaban in Antiphospholipid Syndrome (TRAPS).

In a study of 111 APS patients newly enrolled in RAPS—all with previous venous thromboembolism and on warfarin—Arachchillage et al reported finding significantly higher levels of complement activation markers than in normal controls. At day 42, study patients randomized to rivaroxaban (n=55) had lower levels of complement C3a, C5a and SC5b-9 compared with those who had remained on warfarin. These authors speculate that, in addition to its anticoagulant effect, rivaroxaban may benefit patients with APS by limiting complement activation. [20]

Rituximab can be considered for recurrent thrombosis despite adequate anticoagulation. A nonrandomized prospective study showed rituximab to be effective for noncriteria aPL manifestations (ie, thrombocytopenia and skin ulcers). [5]


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