Autor: |
Jacobs L; Internal Medicine Department, Brugmann University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.; Nephrology and Dialysis Department, Brugmann University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.; Internal Medicine Department, Tivoli University Hospital, Université Libre de Bruxelles, 7100 La Louvière, Belgium., Wauters N; Internal Medicine Department, Tivoli University Hospital, Université Libre de Bruxelles, 7100 La Louvière, Belgium., Lablad Y; Internal Medicine Department, Brugmann University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium., Morelle J; Division of Nephrology, Namur University Hospitals (CHU UCL Namur), 5000 Namur, Belgium., Taghavi M; Internal Medicine Department, Brugmann University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium.; Nephrology and Dialysis Department, Brugmann University Hospital, Université Libre de Bruxelles, 1020 Brussels, Belgium. |
Abstrakt: |
Catastrophic antiphospholipid syndrome (CAPS) is a rare and life-threatening condition characterized by the persistence of antiphospholipid antibodies and occurrence of multiple vascular occlusive events. CAPS currently remains a diagnostic challenge and requires urgent treatment. The diagnosis of CAPS is made difficult by classification criteria used as diagnostic criteria in clinical practice, knowledge derived from retrospective data and case reports, confounding clinical and biological features, and its rapid onset and mortality. The absence of prospective studies of CAPS limits the strength of evidence for guideline treatment protocols. This comprehensive review summarizes the current understanding of the disease, and discusses how the 2023 ACR/EULAR Antiphospholipid Syndrome Classification Criteria impact the definition and therapeutic management of CAPS, which is considered the most severe form of APS. The correct integration of 2023 ACR/EULAR APS classification criteria is poised to facilitate CAPS diagnosis, particularly in critical situations, offering a promising avenue for improved outcomes. |