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Catastrophic antiphospholipid syndrome.

Ronald A Asherson1, Ricard Cervera

  • 1Department of Autoimmune Diseases, Institut Clinic d'Infeccions i Immunologia, Hospital Clinic, Villarroel 170, 08036 Barcelona, Catalonia, Spain.

Current Rheumatology Reports
|September 12, 2003
PubMed
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Catastrophic antiphospholipid syndrome (APS) is a severe condition linked to antiphospholipid antibodies. Prompt identification and aggressive treatment, including anticoagulation, are crucial for managing this life-threatening syndrome, though mortality remains high.

Area of Science:

  • Rheumatology
  • Internal Medicine
  • Hematology

Background:

  • Catastrophic antiphospholipid syndrome (APS) is a rare, life-threatening condition.
  • It is characterized by rapid onset of multiple organ thromboses and/or specific organ damage.
  • APS can occur de novo or as a complication in patients with known antiphospholipid antibody (aPL) syndrome.

Purpose of the Study:

  • To define catastrophic antiphospholipid syndrome (APS).
  • To identify precipitating factors and clinical manifestations.
  • To outline current treatment strategies and outcomes for catastrophic APS.

Main Methods:

  • Literature review and synthesis of existing data on catastrophic APS.
  • Analysis of identified precipitating factors, clinical presentations, and treatment modalities.

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  • Evaluation of reported patient outcomes, including mortality rates.

    • Main Results:

    • Infection is the most common precipitating factor in 55% of catastrophic APS cases.
    • Clinical manifestations include multiorgan failure affecting various unusual vessels and organs.
    • Aggressive treatment with anticoagulation, steroids, IVIg, or plasma exchange is recommended.

    Conclusions:

    • Catastrophic APS requires urgent and aggressive management.
    • Avoiding or treating precipitating factors is essential for prevention.
    • Despite intensive treatment, the mortality rate for catastrophic APS remains high, exceeding 50%.