Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines

  • R A Asherson
    Rheumatic Diseases Unit, University of Cape Town School of Medicine, Cape Town, South Africa
  • R Cervera
    Department of Autoimmune Diseases, Institut Clínic d’Infeccions i Immunologia, Hospital Clínic, Barcelona, Catalonia, Spain,
  • P G de Groot
    Thrombosis and Haemostasis Laboratory, Department of Haematology, University Medical Center Utrecht, Utrecht, The Netherlands
  • D Erkan
    Hospital for Special Surgery, Weill Medical College of Cornell University, New York, USA
  • M-C Boffa
    Department of Internal Medicine, Hôpital Pitié-Salêtrière, Paris, France
  • J-C Piette
    Department of Internal Medicine, Hôpital Pitié-Salêtrière, Paris, France
  • M A Khamashta
    Lupus Unit, The Rayne Institute, St. Thomas’ Hospital, London, UK
  • Y Shoenfeld
    Center for Autoimmune Diseases, Chaim Sheba Medical Center, Tel-Hashomer, Israel

書誌事項

公開日
2003-07
権利情報
  • https://journals.sagepub.com/page/policies/text-and-data-mining-license
DOI
  • 10.1191/0961203303lu394oa
公開者
SAGE Publications

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説明

<jats:p> The term ‘catastrophic’ antiphospholipid syndrome (APS) is used to define an accelerated form of APS resulting in multiorgan failure. Although catastrophicAPS 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 pre-symposium 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 catastrophicAPS must have three clear aims: to treat any precipitating factors (prompt use of antibioticsif 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. </jats:p>

収録刊行物

  • Lupus

    Lupus 12 (7), 530-534, 2003-07

    SAGE Publications

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