Antiphospholipid Syndrome: Pathogenesis, Clinical Manifestations, Diagnosis, and Management Strategies
Suresh Kanna S1, Valarmathy M2, Lakshmi K3, Mani Shanthini4*
Journal of Precision Biosciences 6(1) 1-8 https://doi.org/10.25163/biosciences.612188
Submitted: 16 October 2024 Revised: 16 October 2024 Published: 16 October 2024
Abstract
Background: Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by recurrent thrombosis and/or pregnancy complications associated with the presence of antiphospholipid antibodies (aPL). The condition's rarity, with an incidence of about 5 cases per 100,000 people annually, and its impact on young adults of reproductive age necessitate a detailed understanding of its pathogenesis, clinical manifestations, and management strategies.bMethods: This study conducted a comprehensive review of APS, focusing on its epidemiology, pathogenesis, clinical manifestations, and management. Data were collected from recent literature and clinical guidelines, including studies on aPL positivity, thrombosis, and associated complications. The management strategies for high-risk patients and those with catastrophic APS (CAPS) were also analyzed.bResults: APS manifests primarily through vascular thromboses and pregnancy-related complications. Triple aPL positivity and additional cardiovascular risk factors significantly elevate the risk of severe outcomes. Non-criteria manifestations, such as cardiac valve vegetations and white matter lesions, are managed with aspirin or anticoagulation, while thrombocytopenia is treated based on the underlying cause. CAPS, characterized by rapid thrombotic events and multi-organ failure, requires aggressive treatment including anticoagulation, corticosteroids, IVIG, and plasma exchange. Rituximab is considered for CAPS resistant to standard therapies. Conclusion: Effective management of APS involves tailored anticoagulation therapy, addressing non-criteria manifestations, and aggressive treatment of CAPS. With proper management, patients can achieve favorable outcomes, though vigilance is needed to prevent recurrent events and manage complications. Continued research and individualized care are essential for improving long-term prognosis in APS patients.
Keywords: Antiphospholipid syndrome, Thrombosis, Autoimmune disorder, Pregnancy complications, Anticoagulation therapy
References
Andreoli, L., Chighizola, C. B., Banzato, A., Pons-Estel, G. J., & Meroni, P. L. (2013). Estimated frequency of antiphospholipid antibodies in the general population: A critical review of the literature. Arthritis & Rheumatism, 65(5), 1383-1391.https://doi.org/10.1002/acr.22066
Bertolaccini, M. L., Amengual, O., Andreoli, L., Atsumi, T., Binder, W. L., de Jesus, G. R., ... & Pengo, V. (2013). The evolving classification criteria for the antiphospholipid syndrome. Journal of Thrombosis and Haemostasis, 11(6), 1-10.
Brown, L. (2019). The role of genetics in human behavior. In P. Johnson (Ed.), Advances in behavioral genetics (pp. 45-76). Academic Press.
Davis, P. (2021, November). The future of electric cars. Scientific American, 325(6), 58-65.
Dufrost, V., Reshetnyak, T., Satybaldyeva, M., Gris, J. C., & Sciascia, S. (2019). Antiphospholipid syndrome: A clinical review. Autoimmunity Reviews, 18(8), 791-798.
Foster, K. (2022, April 5). Renewable energy trends on the rise. The New York Times. https://www.nytimes.com/renewable-energy
International Labour Organization. (2019). Global wage report 2018/19: What lies behind gender pay gaps. https://www.ilo.org/wage-report-2018
Jackson, R. (2021, May 12). The importance of mental health awareness. Psychology Today Blog. https://www.psychologytoday.com/mental-health-awareness
Jones, M. (2018, July). Machine learning applications in healthcare. In Proceedings of the 5th International Conference on Data Science (pp. 100-110). Springer.
Kaur, S. (2018). Investigating the impact of air pollution on public health [Master's thesis, University of Toronto]. http://www.tspace.library.utoronto.ca
Legault, K., Schunemann, H. J., Hillis, C., & Akl, E. A. (2021). Antithrombotic therapy in patients with antiphospholipid syndrome: A systematic review and network meta-analysis. The Lancet Rheumatology, 3(2), e92-e104.
Limper, M., de Leeuw, K., & Lely, A. T. (2019). Management of antiphospholipid syndrome during pregnancy. Frontiers in Immunology, 10, 950.
Miyakis, S., Lockshin, M. D., Atsumi, T., Branch, D. W., Brey, R. L., Cervera, R., ... & Wilson, W. A. (2006). International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). Journal of Thrombosis and Haemostasis, 4(2), 295-306.https://doi.org/10.1111/j.1538-7836.2006.01753.x
National Institute of Mental Health. (2020). Depression: Overview and facts. https://www.nimh.nih.gov/depression
National Institute on Drug Abuse. (2020). Drug use in the workplace. https://www.drugabuse.gov/workplace
Nguyen, T. H. (2020). The impact of climate change on coastal ecosystems in Southeast Asia (Doctoral dissertation, University of California, Berkeley). ProQuest Dissertations & Theses Global.
Pengo, V., Banzato, A., Denas, G., Jose, S. P., Padayattil, S. J., Bison, E., & Noventa, F. (2009). Revised criteria for the diagnosis of antiphospholipid syndrome. Journal of Thrombosis and Haemostasis, 7(10), 1737-1740.https://doi.org/10.1111/j.1538-7836.2009.03555.x
Pew Research Center. (2021). Social media use in 2021. https://www.pewresearch.org/social-media-2021
Roberts, T. (2020). Modern techniques in forensic science. Springer.
Ruffatti, A., Tonello, M., Del Ross, T., Cavazzana, A., Noventa, F., & Hoxha, A. (2018). Antiphospholipid syndrome: Refining diagnostic criteria and risk stratification in pregnancy. Journal of Autoimmunity, 90, 1-8.
Ruiz-Irastorza, G., Crowther, M., Branch, W., & Khamashta, M. A. (2010). Antiphospholipid syndrome. The Lancet, 376(9751), 1498-1509.https://doi.org/10.1016/S0140-6736(10)60709-X
Smith, J. A. (2017). Exploring the human mind: Cognitive and behavioral approaches. Oxford University Press.
Spielberg, S. (Director). (1993). Jurassic Park [Film]. Universal Pictures.
Taylor, A. (2015). Data science for dummies (2nd ed.). Wiley. https://www.wiley.com/data-science
TED. (2019, October 20). The future of AI: How artificial intelligence is changing the world. https://www.ted.com
U.S. Department of Health and Human Services. (2020). Healthy People 2020: Progress review. Government Printing Office.
World Health Organization. (2021). Global health statistics: Annual report 2021. https://www.who.int/global-health-statistics
View Dimensions
View Altmetric
Save
Citation
View
Share