Antiphospholipid syndrome: an update for clinicians and scientists

Abstract

Purpose of review Antiphospholipid syndrome (APS) is a leading acquired cause of thrombosis and pregnancy loss. Upon diagnosis (which is not made until at least one morbid event has occurred), anticoagulant medications are typically prescribed in an attempt to prevent future events. This approach is not uniformly effective and does not prevent associated autoimmune and inflammatory complications. The goal of this review is to update clinicians and scientists on mechanistic and clinically-relevant studies from the past 18 months, which have especially focused on inflammatory aspects of APS pathophysiology. Recent findings How antiphospholipid antibodies leverage receptors and signaling pathways to activate cells are being increasingly defined. While established mediators of disease pathogenesis (like endothelial cells and the complement system) continue to receive intensive study, emerging concepts (such as the role of neutrophils) are also receiving increasing attention. In vivo animal studies and small clinical trials are demonstrating how repurposed medications (hydroxychloroquine, statins, rivaroxaban) may have clinical benefit in APS, with these concepts importantly supported by mechanistic data. Summary As anticoagulant medications are not uniformly effective and do not comprehensively target the underlying pathophysiology of APS, there is a continued need to reveal the inflammatory aspects of APS, which may be modulated by novel and repurposed therapies.

Adenosine receptor agonism protects against NETosis and thrombosis in antiphospholipid syndrome

“…Of course, one might ask whether A 2A receptor agonism would benefit other manifestations of APS beyond thrombosis such as pregnancy loss. While dipyridamole has a relatively robust safety record in pregnancy 49 , we would consider such discussions premature, especially given that obstetric APS may have very different pathophysiology from thrombotic APS 50 .…”

Clinical characteristics and prognosis of patients with antiphospholipid antibodies based on cluster analysis: an 8-year cohort study

“…From the clinical perspective, the heart valve disease was progressive despite anticoagulation [ 18 ]. Clinicians should be alert to the underlying SLE in patients with those non-criteria manifestations, for whom anticoagulants alone may offer insufficient protection [ 19 ] and for those with a severe condition immunosuppressive therapy besides anticoagulation may be necessary. Further search is needed to investigate whether non-criteria manifestations can predict future SLE in aPL-positive patients, while it is certain that non-criteria manifestations should be taken into account in the APS assessment [ 20 ].…”

Clinical Characteristics and Prognosis of Patients With Antiphospholipid Antibodies Based on Cluster Analysis: An 8-Year Cohort Study

“…From the clinical perspective, the heart valve disease was progressive despite anticoagulation [18]. Clinicians should be alert to the underlying SLE in patients with those non-criteria manifestations, for whom anticoagulants alone may offer insu cient protection [19] and for those with a severe condition immunosuppressive therapy besides anticoagulation may be necessary. Further search is needed to investigate whether non-criteria manifestations can predict future SLE in aPL-positive patients, while it is certain that non-criteria manifestations should be taken into account in the APS assessment [20].…”