Semin Thromb Hemost
DOI: 10.1055/s-0036-1597282
Review Article
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Management of Thrombotic Antiphospholipid Syndrome

Cecilia Beatrice Chighizola1, 2, Maria Gabriella Raimondo1, 3, Pier Luigi Meroni1, 2, 3
  • 1Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy
  • 2Rheumatology Unit and Experimental laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Milan, Italy
  • 3Department of Rheumatology, ASST Gaetano Pini/CTO, Milan, Italy
Further Information

Publication History

Publication Date:
09 March 2017 (eFirst)

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.