The catastrophic antiphospholipid syndrome in Serbia: diagnostic and management problems

Clin Rev Allergy Immunol. 2009 Jun;36(2-3):98-103. doi: 10.1007/s12016-008-8102-1.

Abstract

Antiphospholipid antibodies (aPL) are a common cause of acquired thrombophilia, termed antiphospholipid syndrome (APS, Huges syndrome). Catastrophic antiphospholipid syndrome (CAPS, Asherson's syndrome) is an unusual form of APS characterized with multi-organ failure and high mortality. Fortunately, CAPS accounts for less than 1% of APS cases. The recurrence rate is low with a stable clinical course if these patients are treated with adequate anticoagulation therapy. Due to the rarity of the condition, an international registry of CAPS patients was created in 2000 supported by the European Forum on Antiphospholipid Antibodies held in Taormina, Italy at the Tenth International Congress on aPL. The objective of our study is to describe characteristics of 12 Serbian patients with CAPS included in the international CAPS registry.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Antibodies, Antiphospholipid / blood
  • Antibodies, Antiphospholipid / immunology*
  • Antiphospholipid Syndrome / diagnosis*
  • Antiphospholipid Syndrome / epidemiology
  • Antiphospholipid Syndrome / physiopathology
  • Antiphospholipid Syndrome / therapy*
  • Catastrophic Illness / therapy
  • Diagnosis, Differential
  • Female
  • Humans
  • Livedo Reticularis / physiopathology
  • Male
  • Microcirculation
  • Middle Aged
  • Multiple Organ Failure / physiopathology
  • Plasmapheresis
  • Purpura, Thrombotic Thrombocytopenic / diagnosis*
  • Registries*
  • Steroids / therapeutic use
  • Thrombosis / physiopathology

Substances

  • Antibodies, Antiphospholipid
  • Steroids