Catastrophic antiphospholipid syndrome: treatment, prognosis, and the risk of relapse

Clin Rev Allergy Immunol. 2009 Jun;36(2-3):80-4. doi: 10.1007/s12016-008-8107-9.

Abstract

The "catastrophic" variant of the antiphospholipid syndrome (APS) is characterized by multiple vascular occlusive events, usually affecting small vessels and developing over a short period of time. Although patients with catastrophic APS represent less than 1% of all patients with APS, they are usually in a life-threatening situation with a 50% mortality rate. The purpose of this paper is to review the treatment strategies and prognostic factors in patients with catastrophic APS. A detailed description of the clinical and laboratory features of the syndrome can be found in the other articles of this issue.

Publication types

  • Review

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use*
  • Age Factors
  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Murine-Derived
  • Antiphospholipid Syndrome / diagnosis*
  • Antiphospholipid Syndrome / immunology
  • Antiphospholipid Syndrome / physiopathology
  • Antiphospholipid Syndrome / therapy*
  • Bacterial Infections / immunology
  • Humans
  • Immunoglobulins, Intravenous / therapeutic use*
  • Immunosuppression Therapy
  • Lupus Erythematosus, Systemic / immunology*
  • Multiple Organ Failure
  • Plasmapheresis
  • Prognosis
  • Recurrence
  • Risk Factors
  • Rituximab
  • Treatment Outcome

Substances

  • Adrenal Cortex Hormones
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunoglobulins, Intravenous
  • Rituximab