Clinical profiles and risk assessment in patients with antiphospholipid antibodies

ABSTRACT

Introduction: Antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilia associated with the presence of persistent antiphospholipid antibodies (aPL). Owing to recent studies, not only APS patients but also incidentally-identified, asymptomatic aPL carriers are able to be stratified in terms of the risk of future thrombotic events, according to the variety and the titer of positive aPL tests and to the non-thrombotic, aPL-associated clinical manifestations.

Areas covered: Here, we critically review (1) criteria manifestations of APS, (2) non-criteria manifestations of APS, (3) risk assessment in patients with APS and in aPL carriers, and (4) the potential role of primary thrombosis prophylaxis in aPL carriers. In addition, we discuss what we are currently able to do and what we need to do in the future for primary prophylaxis against a first thrombotic event.

Expert commentary: We suggest a comprehensive algorithm to stratify thrombotic risk in aPL carriers, including criteria aPL, non-criteria aPL, their scoring systems, and non-criteria manifestations. However, further studies, particularly prospective randomized controlled trials, are highly warranted to establish an effective and tolerable treatment regimen for high risk aPL carriers.

KEYWORDS:

• Antiphospholipid syndrome
• antiphospholipid antibodies
• vascular thrombosis
• pregnancy morbidity
• risk assessment

Declaration of interest

T Atsumi has accepted honoraria for educational meetings from Mitsubishi Tanabe Pharma Co., Chugai Pharmaceutical Co. Ltd., Astellas Pharma Inc., Takeda Pharmaceutical Co. Ltd., Pfizer Inc., AbbVie Inc., Eisai Co. Ltd., Daiichi Sankyo Co. Ltd., Bristol-Myers Squibb Co., UCB Japan Co. Ltd., Eli Lilly Japan K.K. and has received a research grant from Astellas Pharma Inc., Takeda Pharmaceutical Co. Ltd., Mitsubishi Tanabe Pharma Co., Chugai Pharmaceutical Co. Ltd., Daiichi Sankyo Co. Ltd., Otsuka Pharmaceutical Co. Ltd., and Pfizer Inc. Alexion Inc. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.