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Review
. 2024 May 23;13(11):3064.
doi: 10.3390/jcm13113064.

Management of Cardiovascular Complications in Antiphospholipid Syndrome: A Narrative Review with a Focus on Older Adults

Marco Bernardi  1 , Luigi Spadafora  1 , Silvia Andaloro  2 , Alessandra Piscitelli  3 , Giovanni Fornaci  1 , Chiara Intonti  1 , Alberto Emanuele Fratta  3 , Chieh-En Hsu  3 , Karol Kaziròd-Wolski  4 , Theodora Metsovitis  3 , Giuseppe Biondi-Zoccai  5   6 , Pierre Sabouret  7   8 , Emanuele Marzetti  9   10 , Stefano Cacciatore  9
Affiliations
Review

Management of Cardiovascular Complications in Antiphospholipid Syndrome: A Narrative Review with a Focus on Older Adults

Marco Bernardi et al. J Clin Med. .

Abstract

Antiphospholipid syndrome (APS), also known as Hughes syndrome, is an acquired autoimmune and procoagulant condition that predisposes individuals to recurrent thrombotic events and obstetric complications. Central is the role of three types of antiphospholipid antibodies that target phospholipid-binding proteins: lupus anticoagulant (LAC), anti-β2-glycoprotein I (β2-GPI-Ab), and anti-cardiolipin (aCL). Together with clinical data, these antibodies are the diagnostic standard. However, the diagnosis of APS in older adults may be challenging and, in the diagnostic workup of thromboembolic complications, it is an underestimated etiology. The therapeutic management of APS requires distinguishing two groups with differential risks of thromboembolic complications. The standard therapy is based on low-dose aspirin in the low-risk group and vitamin K antagonists in the high-risk group. The value of direct oral anticoagulants is currently controversial. The potential role of monoclonal antibodies is investigated. For example, rituximab is currently recommended in catastrophic antiphospholipid antibody syndrome. Research is ongoing on other monoclonal antibodies, such as daratumumab and obinutuzumab. This narrative review illustrates the pathophysiological mechanisms of APS, with a particular emphasis on cardiovascular complications and their impact in older adults. This article also highlights advancements in the diagnosis, risk stratification, and management of APS.

Keywords: anti-cardiolipin antibodies; anti-β2-glycoprotein I antibodies; lupus anticoagulant; miRNA; monoclonal antibodies; stroke; thrombosis.

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Conflict of interest statement

G.B.-Z. has consulted for Amarin, Balmed, Cardionovum, Crannmedical, Endocore Lab, Eukon, Guidotti, Innovheart, Meditrial, Microport, Opsens Medical, Terumo, and Translumina, outside of the present work. P.S. has received speaker fees from AstraZeneca, Amgen, Axis TV, BMS, Les laboratoires Servier, Novartis, Novonordisk, Sanofi, and Vifor, outside of the present work. E.M. has received speaker fees from Abbot, Difass International, Nestlè, and Nutricia and consulting fees from Cepton Strategies and Pfizer, outside of the present work. All other authors report no conflicts of interest.

Figures

Figure 1
Figure 1
Clinical manifestations of antiphospholipid syndrome. Created with BioRender.com (accessed on 2 May 2024).
Figure 2
Figure 2
Recommended treatment approaches according to the latest recommendations by the European League Against Rheumatism (EULAR). In case of recurrent thrombosis despite anticoagulant therapy in the therapeutic range, it is recommended to increase the INR in the 3–4 range. Abbreviations: APS, antiphospholipid syndrome; INR, international normalized ratio; VKA, vitamin K antagonist. Created with BioRender.com (accessed on 2 May 2024).
See this image and copyright information in PMC

References

    1. Miyakis S., Lockshin M.D., Atsumi T., Branch D.W., Brey R.L., Cervera R., Derksen R.H., De Groot P.G., Koike T., Meroni P.L., et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS) J. Thromb. Haemost. 2006;4:295–306. doi: 10.1111/j.1538-7836.2006.01753.x. - DOI - PubMed
    1. Knight J.S., Kanthi Y. Mechanisms of immunothrombosis and vasculopathy in antiphospholipid syndrome. Semin. Immunopathol. 2022;44:347–362. doi: 10.1007/s00281-022-00916-w. - DOI - PMC - PubMed
    1. Dabit J.Y., Valenzuela-Almada M.O., Vallejo-Ramos S., Duarte-Garcia A. Epidemiology of Antiphospholipid Syndrome in the General Population. Curr. Rheumatol. Rep. 2022;23:8510. doi: 10.1007/s11926-021-01038-2. - DOI - PMC - PubMed
    1. Cervera R., Serrano R., Pons-Estel G.J., Ceberio-Hualde L., Shoenfeld Y., de Ramon E., Buonaiuto V., Jacobsen S., Zeher M.M., Tarr T., et al. Morbidity and mortality in the antiphospholipid syndrome during a 10-year period: A multicentre prospective study of 1000 patients. Ann. Rheum. Dis. 2015;74:1011–1018. doi: 10.1136/annrheumdis-2013-204838. - DOI - PubMed
    1. Espinosa G., Bucciarelli S., Asherson R.A., Cervera R. Morbidity and mortality in the catastrophic antiphospholipid syndrome: Pathophysiology, causes of death, and prognostic factors. Semin. Thromb. Hemost. 2008;34:290–294. doi: 10.1055/s-0028-1082274. - DOI - PubMed

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