Novel diagnostic and therapeutic frontiers in thrombotic anti-phospholipid syndrome

Intern Emerg Med. 2017 Feb;12(1):1-7. doi: 10.1007/s11739-016-1596-2. Epub 2017 Jan 2.

Abstract

The anti-phospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity, associated with a persistent positivity for anti-phospholipid antibodies (aPL). The current classification criteria for APS include three laboratory tests: lupus anti-coagulant (LA), anti-cardiolipin (aCL), and anti-β2 glycoprotein-I (β2GPI). To date, the therapeutic approach for thrombotic APS mainly centers on long-term anti-coagulation with a vitamin K antagonist (VKA). APS management may represent a challenge for the treating physicians. Patients with different aPL profiles need a tailored risk-stratified approach. Moreover, in patients with recurrent thrombotic events despite therapy with VKA, or in those with microvascular involvement, new therapeutic options are highly needed. In this review, we aim to elucidate recent findings about new aPL specifities, available risk scoring models, and novel therapeutic approaches in APS management.

Keywords: APS; Pregnancy morbidity; Thrombosis; aPL.

Publication types

  • Review

MeSH terms

  • Adult
  • Antibodies, Anticardiolipin / analysis
  • Antibodies, Anticardiolipin / blood
  • Antibodies, Monoclonal / pharmacology
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized / pharmacology
  • Antibodies, Monoclonal, Humanized / therapeutic use
  • Anticoagulants / pharmacology
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / diagnosis*
  • Antiphospholipid Syndrome / therapy*
  • Female
  • Fibrinolytic Agents / pharmacology
  • Fibrinolytic Agents / therapeutic use
  • Humans
  • Hydroxychloroquine / pharmacology
  • Hydroxychloroquine / therapeutic use
  • Lupus Coagulation Inhibitor / analysis
  • Lupus Coagulation Inhibitor / blood
  • Pregnancy
  • Pregnancy Complications / diagnosis
  • Pregnancy Complications / prevention & control
  • Pregnancy Complications / therapy
  • Risk Assessment / methods
  • Rivaroxaban / pharmacology
  • Rivaroxaban / therapeutic use
  • Thrombosis / diagnosis
  • Thrombosis / therapy*
  • beta 2-Glycoprotein I / analysis
  • beta 2-Glycoprotein I / blood

Substances

  • Antibodies, Anticardiolipin
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • Anticoagulants
  • Fibrinolytic Agents
  • Lupus Coagulation Inhibitor
  • beta 2-Glycoprotein I
  • Hydroxychloroquine
  • Rivaroxaban
  • eculizumab