• Z Amoura, B Bader-Meunier, C Bal Dit Sollier, A Belot, Y Benhamou, H Bezanahary, F Cohen, N Costedoat-Chalumeau, L Darnige, L Drouet, E Elefant, A Harroche, M Lambert, T Martin, I Martin-Toutain, A Mathian, A Mekinian, M Pineton De Chambrun, L de Pontual, D Wahl, C Yelnik, S Zuily, and Collaborators.
• Department of Internal Medicine, Institut E3M, CIMI-Paris, Faculty of medicine, National Reference Centre of Systemic Lupus, antiphospholipid syndrome, and other autoimmune diseases, Sorbonne University, AP-HP, Pitié Salpêtrière, boulevard de l'Hôpital, 75013 Paris, France. Electronic address: Zahir.amoura@aphp.fr.
• Rev Med Interne. 2023 Sep 1; 44 (9): 495520495-520.

AbstractAntiphospholipid syndrome (APS) is a chronic autoimmune disease involving vascular thrombosis and/or obstetric morbidity and persistent antibodies to phospholipids or certain phospholipid-associated proteins. It is a rare condition in adults and even rarer in children. The diagnosis of APS can be facilitated by the use of classification criteria based on a combination of clinical and biological features. APS may be rapidly progressive with multiple, often synchronous thromboses, resulting in life-threatening multiple organ failure. This form is known as "catastrophic antiphospholipid syndrome" (CAPS). It may be primary or associated with systemic lupus erythematosus (associated APS) and in very rare cases with other systemic autoimmune diseases. General practitioners and paediatricians may encounter APS in patients with one or more vascular thromboses. Because APS is so rare and difficult to diagnosis (risk of overdiagnosis) any suspected case should be confirmed rapidly and sometimes urgently by an APS specialist. First-line treatment of thrombotic events in APS includes heparin followed by long-term anticoagulation with a VKA, usually warfarin. Except in the specific case of stroke, anticoagulants should be started as early as possible. Any temporary discontinuation of anticoagulants is associated with a high risk of thrombosis in APS. A reference/competence centre specialised in autoimmune diseases must be urgently consulted for the therapeutic management of CAPS.Copyright © 2023 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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