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Lupus, Vol. 12, No. 7,
530-534 (2003)
DOI: 10.1191/0961203303lu394oa
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, rcervera{at}clinic.ub.es
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
Catastrophic Antiphospholipid Syndrome Registry Project Group
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.
Key Words: anticardiolipin antibodies • antiphospholipid antibodies • catastrophic antiphospholipid syndrome • lupus anticoagulant • plasma exchange
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