Therapeutic and prognostic considerations in catastrophic antiphospholipid syndrome

Autoimmun Rev. 2006 Dec;6(2):98-103. doi: 10.1016/j.autrev.2006.06.013. Epub 2006 Jul 21.

Abstract

Catastrophic antiphospholipid syndrome (CAPS) is a rapidly progressive life-threatening disease that causes multiple organ thromboses in the presence of antiphospholipid antibodies. High index of clinical suspicion and careful investigation are required to make an early diagnosis so that treatment with anticoagulation, corticosteroids, and plasma exchange or intravenous immunoglobulins can be initiated. Despite this multi-modal treatment, CAPS is associated with high mortality; evidence-based management recommendations do not exist due to the rarity of the condition and the lack of controlled studies. This article reviews the therapeutic and prognostic controversies that were addressed during the 1st International Symposium on CAPS.

Publication types

  • Review

MeSH terms

  • Animals
  • Anticoagulants / therapeutic use
  • Antiphospholipid Syndrome / therapy*
  • Catastrophic Illness / therapy
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use
  • Plasma Exchange
  • Plasmapheresis
  • Prognosis
  • Protein C / therapeutic use

Substances

  • Anticoagulants
  • Immunoglobulins, Intravenous
  • Protein C