Cecilia Beatrice Chighizola1,2,3, Pier Luigi Meroni4. 1. Department of Clinical Sciences and Community Health, University of Milan, Via Festa del Perdono 7, 20122, Milan, Italy. cecilia.chighizola@unimi.it. 2. Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, 20095, Cusano Milanino, Milan, Italy. cecilia.chighizola@unimi.it. 3. Unit of Immunology, Allergology and Rheumatology, IRCCS Istituto Auxologico Italiano, Piazzale Brescia 20, 20149, Milan, Italy. cecilia.chighizola@unimi.it. 4. Experimental Laboratory of Immunological and Rheumatologic Researches, IRCCS Istituto Auxologico Italiano, Via Zucchi 18, 20095, Cusano Milanino, Milan, Italy.
Abstract
PURPOSE OF REVIEW: The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years. RECENT FINDINGS: Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives. Several drugs have been proposed as effective additional tools for the management of thrombotic APS: hydroxychloroquine, statins, vitamin D, and sirolimus might be beneficial when added on the top of anticoagulation. Pregnant women with thrombotic APS should be switched to low-dose aspirin plus low molecular weight heparin at therapeutic dose. Despite adequate treatment, APS patients display a significant rate of recurrences; rituximab, eculizumab, and intravenous immunoglobulins are among the options to be considered for these patients. From 2013 to date, the kaleidoscope of therapeutic options in thrombotic APS has been enriched, but tangible improvements in the management of patients are still awaited.
PURPOSE OF REVIEW: The aim of this review is to provide an update of the therapeutic tools for thrombotic anti-phospholipid syndrome (APS), focusing on the last 5 years. RECENT FINDINGS: Early studies appointed anticoagulation at moderate intensity as the mainstay of treatment of thrombotic APS; in the last 5 years, the strategy has not much mutated. Some uncertainties regarding the role of direct oral anticoagulants and the optimal regimen for arterial thrombotic APS still persist: high-intensity anticoagulation, anticoagulation plus anti-platelet agent, and double anti-platelet agents being the possible alternatives. Several drugs have been proposed as effective additional tools for the management of thrombotic APS: hydroxychloroquine, statins, vitamin D, and sirolimus might be beneficial when added on the top of anticoagulation. Pregnant women with thrombotic APS should be switched to low-dose aspirin plus low molecular weight heparin at therapeutic dose. Despite adequate treatment, APSpatients display a significant rate of recurrences; rituximab, eculizumab, and intravenous immunoglobulins are among the options to be considered for these patients. From 2013 to date, the kaleidoscope of therapeutic options in thrombotic APS has been enriched, but tangible improvements in the management of patients are still awaited.
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