Catastrophic antiphospholipid syndrome

Authors

DOI:

https://doi.org/10.35954/SM2026.45.1.7.e501

Keywords:

Antigoagulants, antibodies antiphospholipid, adrenal cortex hormones, autoimmune diseases, inflammation, multiple organ failure, antiphospholipid syndrome, thrombosis

Abstract

Introduction: Catastrophic Antiphospholipid Syndrome is a rare variant of Antiphospholipid Syndrome, but it is highly significant due to its poor prognosis and high mortality rate, which approaches 40%. Its pathogenesis involves thrombotic and inflammatory mechanisms associated with the presence of antiphospholipid antibodies, affecting multiple organs simultaneously or within a short period of time. This leads to multiorgan failure, and therefore, treatment is aimed at attenuating the inflammatory response and preventing new thrombotic events.

Objective: To highlight the importance of recognizing the clinical manifestations of this rare disease in order to initiate early therapeutic measures and increase the survival probability of affected patients.

Methodology: The clinical case of a patient treated at the Central Hospital of the Armed Forces was analyzed. A literature review was conducted, including published articles, case reports, and clinical cases from the past 20 years, in Spanish, English, and Portuguese. No clinical cases or studies related to Catastrophic Antiphospholipid Syndrome in Uruguay were found, except during pregnancy. The following databases were consulted: Elsevier, Scielo, PubMed, MSD, and the SER textbook.

The descriptors used were: anticoagulant; antiphospholipid antibodies; corticosteroids; autoimmune diseases; inflammation; multiorgan failure; antiphospholipid syndrome; thrombosis.

Discussion: Given that Catastrophic Antiphospholipid Syndrome is a rare variant but with a poor prognosis and high mortality rate, it is crucial to promptly recognize the diagnosis and initiate intensive treatment in a timely manner. This can alter both the functional and vital prognosis of these patients.

This article was approved by the Editorial Committee.

Received for review: September 2025.
Accepted for publication:
December 2025.
Publication date:
January 2026.
Correspondence:
Armed Forces Central Hospital. Av. Centenario 3057. Postal code 11600. Tel.: (+598) 2487 66 66. Montevideo, Uruguay.
Contact email: 
 lorena.debernardis@gmail.com      

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Author Biographies

Maria Lorena Debernardis, Hospital Central de las Fuerzas Armadas

Internist. National Directorate of Health of the Armed Forces. Central Hospital of the Armed Forces. Emergency Department. CONTRIBUTED TO THE MANUSCRIPT IN: conception, data acquisition and analysis, interpretation and discussion of results, writing and approval of the final version.

Maria Alejandra de Vargas Moraes , Hospital Central de las Fuerzas Armadas

Internal medicine physician. National Directorate of Health of the Armed Forces. Central Hospital of the Armed Forces. Emergency Department. CONTRIBUTED TO THE MANUSCRIPT IN: Design, data acquisition and analysis, interpretation and discussion of results, writing.

María Lucrecia Martin, Hospital Central de las Fuerzas Armadas

Graduate in Nursing. National Directorate of Health of the Armed Forces. Central Hospital of the Armed Forces. Haematology ward. CONTRIBUTED TO THE MANUSCRIPT BY: Interpreting and discussing results, writing, critical review.

Ana Karen Yedrzejewski , Hospital Central de las Fuerzas Armadas

Internist. Internist. National Directorate of Health of the Armed Forces. Central Hospital of the Armed Forces. Emergency Department. CONTRIBUTED TO THE MANUSCRIPT BY: Interpreting and discussing results, writing, critical review.

References

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Barbhaiya M, Zuily S, Naden R, Hendry A, Manneville F, Amigo MC, et al. 2023 ACR/EULAR antiphospholipid syndrome classification criteria. Ann Rheum Dis. 2023 Oct; 82(10):1258-70. https://doi.org/10.1136/ard-2023-224609 Epub 2023 Aug 28. PMID: 37640450. DOI: https://doi.org/10.1136/ard-2023-224609

Asherson RA, Cervera R, de Groot PG, Erkan D, Boffa MC, Piette JC, et al. Catastrophic antiphospholipid syndrome: international consensus statement on classification criteria and treatment guidelines. Lupus 2003; 12(7):530-4. https://doi.org/10.1191/0961203303lu394oa PMID: 12892393. DOI: https://doi.org/10.1191/0961203303lu394oa

Costedoat-Chalumeau N, Arnaud L, Saadoun D, Chastre J, Leroux G, Cacoub P, et al. Le syndrome catastrophique des antiphospholipides [Catastrophic antiphospholipid syndrome]. Rev Med Interne. 2012 Apr; 33(4):19499. https://doi.org/10.1016/j.revmed.2012.01.005 Epub 2012 Feb 16. PMID: 22341856. DOI: https://doi.org/10.1016/j.revmed.2012.01.005

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Rossi A. Síndrome antifosfolípido catastrófico (SAFC). Hematología 2014 Ene‑Abr; 18(1):40‑7. Disponible en: https://www.sah.org.ar/revistasah/numeros/Vol18.n1.40-47.pdf [Consulta 21/07/2025].

Published

2026-01-29

How to Cite

1.
Debernardis ML, de Vargas Moraes MA, Martin ML, Yedrzejewski AK. Catastrophic antiphospholipid syndrome . Salud mil [Internet]. 2026 Jan. 29 [cited 2026 May 31];45(1):e501. Available from: https://revistasaludmilitar.uy/ojs/index.php/Rsm/article/view/476

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