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Catastrophic antiphospholipid syndrome.

R A Asherson1, R Cervera

  • 1Department of Medicine, The Groote School Hospital, University of Cape Town School of Medicine, South Africa. ashspot@icon.co.za

Current Opinion in Hematology
|August 29, 2000
PubMed
Summary
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Antiphospholipid syndrome involves blood clots and pregnancy loss. Catastrophic antiphospholipid syndrome is a severe variant affecting small vessels, requiring aggressive treatment including anticoagulation and corticosteroids.

Area of Science:

  • Rheumatology
  • Hematology
  • Immunology

Background:

  • Antiphospholipid syndrome (APS) classically presents with thrombosis and/or fetal loss, linked to antiphospholipid antibodies.
  • A severe variant, catastrophic APS (CAPS), primarily affects small vessels, leading to multi-organ damage.
  • CAPS is associated with marked thrombocytopenia, microangiopathic anemia, and high mortality.

Purpose of the Study:

  • To differentiate between classic and catastrophic antiphospholipid syndrome.
  • To outline the distinct clinical features and diagnostic criteria for CAPS.
  • To detail the recommended treatment strategies for catastrophic APS.

Main Methods:

  • Review of clinical presentations and laboratory findings in APS and CAPS.
  • Analysis of diagnostic markers including anticardiolipin antibodies and lupus anticoagulant.

Related Experiment Videos

  • Evaluation of treatment outcomes for patients with catastrophic APS.

    • Main Results:

    • Classic APS involves major vessel thrombosis and fetal loss.
    • Catastrophic APS is characterized by small vessel thrombosis, severe thrombocytopenia, and organ damage.
    • Mortality rates for CAPS approach 50% in reported cases.

    Conclusions:

    • Distinguishing between classic and catastrophic APS is crucial for appropriate management.
    • Aggressive multi-modal treatment is essential for CAPS, including anticoagulation, corticosteroids, and plasmapheresis.
    • Early recognition and intervention in CAPS can potentially improve patient outcomes.