The antiphospholipid syndrome: still an enigma

Area of Science:

• Immunology
• Hematology
• Obstetrics

Background:

• Antiphospholipid syndrome (APS) is characterized by thrombosis and/or pregnancy morbidity in patients with antiphospholipid antibodies (aPL).
• aPL are a leading cause of acquired thrombophilia, linked to both venous and arterial thrombosis.
• The precise prothrombotic mechanisms of aPL remain unclear due to their heterogeneity and potential impact on multiple coagulation pathways.

Purpose of the Study:

• To review the current understanding of antiphospholipid syndrome (APS) pathogenesis, diagnosis, and management.
• To highlight the heterogeneity of antiphospholipid antibodies (aPL) and their targets, such as β2-glycoprotein 1 (β2GPI) and prothrombin.
• To discuss diagnostic criteria and current therapeutic strategies, including anticoagulation and emerging treatments.

Main Methods:

• Review of existing clinical and basic research on antiphospholipid syndrome (APS) and antiphospholipid antibodies (aPL).
• Analysis of diagnostic methods for aPL, including lupus anticoagulant (LA), anticardiolipin, and anti-β2-glycoprotein 1 antibodies.
• Evaluation of current and emerging therapeutic approaches for thrombotic and obstetrical APS.

Main Results:

• Antiphospholipid antibodies (aPL) target proteins like β2GPI and prothrombin, contributing to acquired thrombophilia.
• Diagnosis relies on detecting lupus anticoagulant (LA) or specific aPL antibodies.
• Current mainstay therapy for thrombotic APS is indefinite anticoagulation; new strategies and caution with DOACs are noted.

Conclusions:

• A unified mechanism for aPL prothrombotic activity is not yet defined.
• There is a need for better understanding of APS pathogenesis, risk stratification, and targeted treatments.
• Current treatments include anticoagulation for thrombotic APS and aspirin/LMWH for obstetrical APS, with ongoing research for improved outcomes.