• Ignasi Rodriguez-Pintó, Gerard Espinosa, and Ricard Cervera.
• Department of Autoimmune Diseases, Reference Centre for Systemic Autoimmune Diseases (UEC, CSUR) of the Catalan and Spanish Health Systems/Member of ERN-ReCONNET, Hospital Clínic, Barcelona, Catalonia, Spain.
• Med Clin (Barc). 2024 Aug 1; 163 Suppl 1: S31S35S31-S35.

AbstractThe catastrophic antiphospholipid syndrome (CAPS) is a rare life-threatening clinical condition that represents the most severe clinical presentation of the antiphospholipid syndrome (APS). It was first described in 1992 in a group of patients that presented with multiorgan involvement and microangiopathic features of APS. Most of the current knowledge of CAPS comes from the analysis of all cases collected at the "CAPS Registry" that was created in 2000 to perform studies on this condition. Most cases are triggered by a prothrombotic situation that leads to a multiorgan thrombosis and a cytokine storm. The analysis of cases included in the "CAPS Registry" has shown that the triple therapy with anticoagulation, glucocorticoids, and plasma exchange and/or intravenous immunoglobulins is associated to a better prognosis of CAPS. The improvement of the knowledge allowed a decrease from the 50% mortality rate reported in the first series to 25-30% in the most recent publications.Copyright © 2024 Elsevier España, S.L.U. All rights reserved.

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