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[Catastrophic antiphospholipid syndrome].

N Costedoat-Chalumeau1, L Arnaud, D Saadoun

  • 1Service de médecine interne, centre de référence national pour le lupus systémique et le syndrome des antiphospholipides, faculté Paris 6, hôpital Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75013 Paris, France. nathalie.costedoat@gmail.com

La Revue De Medecine Interne
|February 21, 2012
PubMed
Summary
This summary is machine-generated.

Catastrophic antiphospholipid syndrome (CAPS) is a rare, severe condition involving widespread clots. Early diagnosis and treatment significantly improve patient outcomes, reducing mortality rates.

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Area of Science:

  • Vascular Medicine
  • Immunology
  • Hematology

Context:

  • Catastrophic antiphospholipid syndrome (CAPS) is a rare but severe thrombotic event.
  • It affects the microvasculature, leading to rapid multiorgan failure.
  • CAPS occurs in less than 1% of antiphospholipid syndrome patients.

Purpose:

  • To summarize the clinical presentation, precipitating factors, and management of CAPS.
  • To highlight the importance of early diagnosis and treatment in improving outcomes.
  • To discuss differential diagnoses and preventative strategies.

Summary:

  • CAPS involves widespread thrombosis in small blood vessels, presenting with multiorgan failure.
  • Precipitating factors include infections, surgery, or anticoagulation withdrawal.
  • Treatment involves anticoagulation, corticosteroids, and potentially plasma exchange or IVIg.

Impact:

  • CAPS mortality has decreased, with hospital-specific rates reduced to 10%.
  • Effective management strategies are crucial for patient survival and prognosis.
  • Prevention focuses on managing infections and perioperative care in APS patients.