Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines Conference Paper uri icon


MeSH Major

  • Antiphospholipid Syndrome
  • Multiple Organ Failure


  • The term 'catastrophic' antiphospholipid syndrome (APS) is used to define an accelerated form of APS resulting in multiorgan failure. Although catastrophic APS patients represent less than 1% of all patients with APS, they are usually in a life-threatening medical situation that requires high clinical awareness. The careful and open discussion of several proposals by all participants in the presymposium workshop on APS consensus, held in Taormina on occasion of the 10th International Congress on aPL and chaired by Munther A Khamashta and Yehuda Shoenfeld (29 September 2002), has allowed the acceptation of a preliminary set of classification criteria. On the other hand, the optimal management of catastrophic APS must have three clear aims: to treat any precipitating factors (prompt use of antibiotics if infection is suspected, amputation for any necrotic organ, high awareness in patients with APS who undergo an operation or an invasive procedure), to prevent and to treat the ongoing thrombotic events and to suppress the excessive cytokine 'storm'. Anticoagulation (usually intravenous heparin followed by oral anticoagulants), corticosteroids, plasma exchange, intravenous gammaglobulins and, if associated with lupus flare, cyclophosphamide, are the most commonly used treatments for catastrophic APS patients.

publication date

  • August 2003



  • Conference Paper



  • eng

Digital Object Identifier (DOI)

  • 10.1191/0961203303lu394oa

PubMed ID

  • 12892393

Additional Document Info

start page

  • 530

end page

  • 4


  • 12


  • 7