Catastrophic antiphospholipid (Asherson's) syndrome and genetic thrombophilic disorders in obstetrics

Autoimmun Rev. 2006 Dec;6(2):89-93. doi: 10.1016/j.autrev.2006.06.011. Epub 2006 Jul 24.

Abstract

Catastrophic antiphospholipid syndrome (CAPS) (Asherson's Syndrome), is a life-threatening condition characterized by a rapidly progressive thromboses resulting in a multiorgan dysfunction syndrome (MODS), evidence of systemic inflammatory response syndrome (SIRS) in the presence of antiphospholipid antibodies. CAPS differs from the classic APS by predominantly affecting small vessels, involvement of unusual organs, rapid onset of MODS, and the development of acute respiratory distress syndrome (ARDS) in 25% of patients, which is a feature of SIRS. Obstetric-related multiorgan failure may be a feature of a subset of CAPS more frequently than was previously thought. Patients with obstetric complications should be tested for antiphospholipid antibodies and genetic thrombophilia in order to institute early prophylaxis. Low-molecular-weight heparin is the drug of choice for preventing obstetric complications and CAPS due to its anticoagulant and anti-inflammatory properties.

Publication types

  • Review

MeSH terms

  • Antiphospholipid Syndrome / etiology
  • Antiphospholipid Syndrome / genetics*
  • Antiphospholipid Syndrome / physiopathology*
  • Catastrophic Illness
  • Female
  • Humans
  • Pregnancy
  • Pregnancy Complications, Hematologic / genetics*
  • Pregnancy Complications, Hematologic / physiopathology*
  • Thrombophilia / genetics*
  • Thrombophilia / physiopathology*