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Antiphospholipid syndrome (APS) involves antiphospholipid antibodies (aPL) linked to blood clots and pregnancy loss. Current criteria are debated, emphasizing a risk-stratified approach for management.

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Area of Science:

  • Autoimmune disorders
  • Immunology
  • Vascular medicine

Background:

  • Antiphospholipid syndrome (APS) is an autoimmune disorder defined by antiphospholipid antibodies (aPL), thrombosis, and/or pregnancy loss.
  • Classification criteria were established in 2005, but vascular and obstetric manifestations have distinct pathogenetic mechanisms.
  • Thrombosis is linked to procoagulative changes (second-hit theory), while pregnancy morbidity involves placental thrombosis and complement activation.

Purpose of the Study:

  • To review the current understanding of antiphospholipid syndrome (APS) and its diagnostic criteria.
  • To discuss the role of antiphospholipid antibodies (aPL) in thrombosis and pregnancy complications.
  • To highlight the need for a risk-stratified approach in managing APS patients.

Main Methods:

  • Review of existing literature and consensus criteria for Antiphospholipid Syndrome (APS).
  • Analysis of pathogenetic mechanisms underlying vascular and obstetric manifestations.
  • Evaluation of laboratory diagnostic tests, including Lupus Anticoagulant (LA) and ELISA-based assays for anticardiolipin (aCL) and anti-β2-glycoprotein I antibodies.

Main Results:

  • Significant interlaboratory discrepancies exist in ELISA tests for aPL, despite relatively standardized LA testing.
  • The precise contribution of aPL to thrombosis and the validity of current APS criteria are under discussion.
  • Different pathogenetic mechanisms for vascular and obstetric APS suggest potential variations in diagnostic criteria.

Conclusions:

  • Antiphospholipid syndrome (APS) management requires a risk-stratified approach, considering aPL profile, cardiovascular risks, and associated autoimmune diseases.
  • Prophylaxis strategies (intensity and duration) should be individualized based on patient-specific factors.
  • Further recommendations are anticipated from leading experts following recent international congresses.