Is there a role for immunosuppression in antiphospholipid syndrome?
- PMID: 31808842
- PMCID: PMC6913487
- DOI: 10.1182/hematology.2019000073
Is there a role for immunosuppression in antiphospholipid syndrome?
Abstract
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by thrombosis, pregnancy morbidity, or nonthrombotic manifestations in patients with persistently positive antiphospholipid antibodies (aPL). Conventional APS treatment focuses on antithrombotic strategies, which are usually ineffective for the microvascular and nonthrombotic manifestations of aPL. Using a case-based presentation, this review focuses on the role of immunosuppression in nonobstetric APS, including B-cell inhibition (rituximab, belimumab, and bortezomib), complement inhibition (eculizumab), mechanistic target of rapamycin inhibition (sirolimus), vascular endothelial cell modulation (defibrotide), statins, and traditional rheumatologic disease-modifying agents (hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide).
© 2019 by The American Society of Hematology. All rights reserved.
Conflict of interest statement
Conflict-of-interest disclosure: D.E. received research grants from American College of Rheumatology (ACR), European League Against Rheumatism (EULAR), Lupus Clinical Trials Consortium, and National Institutes of Health; is a clinical research investigator for GSK-sponsored studies; and is a consultant for GSK, UCB, and Exagen. E.S. and R.W. declare no competing financial interests.
References
-
- Miyakis S, Lockshin MD, Atsumi T, et al. . International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006;4(2):295-306. - PubMed
-
- Legault K, Schunemann H, Hillis C, et al. . McMaster RARE-Bestpractices clinical practice guideline on diagnosis and management of the catastrophic antiphospholipid syndrome. J Thromb Haemost. 2018;16(8):1656-1664. - PubMed
-
- Garcia D, Erkan D. Diagnosis and management of the antiphospholipid syndrome. N Engl J Med. 2018;378(21):2010-2021. - PubMed
-
- Erkan D, Yazici Y, Peterson MG, Sammaritano L, Lockshin MD. A cross-sectional study of clinical thrombotic risk factors and preventive treatments in antiphospholipid syndrome. Rheumatology (Oxford). 2002;41(8):924-929. - PubMed
-
- Willis R, Cohen H, Giles I, et al. . Mechanisms of antiphospholipid antibody-mediated thrombosis. In: Erkan D, Lockshin MD, eds. Antiphospholipid Syndrome Current Research Highlights and Clinical Insights. New York: Springer; 2017:77-116.
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