What we know and what we don't know about catastrophic antiphospholipid syndrome.
| Autor: | Rodriguez-Pintó I; Autoimmune Diseases Unit, Hospital Universitari Mútua de Terrassa, Terrassa, Catalonia, Spain., Espinosa G; 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., Cervera R; 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. |
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| Jazyk: | angličtina |
| Zdroj: | Rheumatology (Oxford, England) [Rheumatology (Oxford)] 2024 Feb 06; Vol. 63 (SI), pp. SI46-SI53. |
| DOI: | 10.1093/rheumatology/kead556 |
| Abstrakt: | Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition. (© The Author(s) 2024. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissions@oup.com.) |
| Databáze: | MEDLINE |
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