• Pineton de ChambrunMarcMSorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-phospholipides, Romaric Larcher, Frédéric Pène, Laurent Argaud, Alexandre Demoule, Matthieu Jamme, Remi Coudroy, Alexis Mathian, Aude Gibelin, Elie Azoulay, Yacine Tandjaoui-Lambiotte, Auguste Dargent, François-Michel Beloncle, Jean-Herlé Raphalen, Amélie Couteau-Chardon, Nicolas de Prost, Jérôme Devaquet, Damien Contou, Samuel Gaugain, Pierre Trouiller, Steven Grangé, Stanislas Ledochowski, Jérémie Lemarie, Stanislas Faguer, Vincent Degos, Alain Combes, Charles-Edouard Luyt, Zahir Amoura, and SAPHIR study group.
• Sorbonne Université, Assistance Publique-Hôpitaux de Paris (APHP), Hôpital La Pitié-Salpêtrière, Institut E3M, Service de Médecine Interne 2, Centre de Référence National Lupus Systémique, Syndrome des Anticorps Anti-phospholipides et autres maladies auto-immunes systémiques rares, Paris, France; Sorbonne Université, APHP, Hôpital La Pitié-Salpêtrière, Institut de Cardiométabolisme et Nutrition (ICAN), Service de Médecine Intensive-Réanimation, Paris, France. Electronic address: marc.dechambrun@gmail.com.
• J. Autoimmun. 2019 Sep 1; 103: 102292.

PurposeCatastrophic antiphospholipid syndrome (CAPS), the most severe manifestation of antiphospholipid syndrome (APS), is characterised by simultaneous thromboses in multiple organs. Diagnosing CAPS can be challenging but its early recognition and management is crucial for a favourable outcome. This study was undertaken to evaluate the frequencies, distributions and ability to predict mortality of "definite/probable" or "no-CAPS" categories of thrombotic APS patients requiring admission to the intensive care unit (ICU).MethodsThis French national multicentre retrospective study, conducted from January 2000 to September 2018, included all APS patients with any new thrombotic manifestation(s) admitted to 24 ICUs.ResultsOne hundred and thirty-four patients (male/female ratio: 0.4; mean age at admission: 45.4 ± 15.0 years), who experienced 152 CAPS episodes, required ICU admission. The numbers of definite, probable or no-CAPS episodes, respectively, were: 11 (7.2%), 60 (39.5%) and 81 (53.3%). No histopathological proof of microvascular thrombosis was the most frequent reason for not being classified as definite CAPS. Overall, 35/152 (23.0%) episodes were fatal, with comparable rates for definite/probable CAPS and no CAPS (23% vs. 28.8% respectively, p = 0.4). The Kaplan-Meier curve of estimated probability of survival showed no between-group survival difference (log-rank test p = 0.5).ConclusionsIn this study, CAPS criteria were not associated with mortality of thrombotic APS patients requiring ICU admission. Further studies are need evaluate the adequacy of CAPS criteria for critically-ill APS patients.Copyright © 2019 Elsevier Ltd. All rights reserved.

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