Abstract
The anti-phospholipid syndrome (APS) is an autoimmune disorder characterized by vascular thrombosis and/or pregnancy morbidity, associated with a persistent positivity for anti-phospholipid antibodies (aPL). The current classification criteria for APS include three laboratory tests: lupus anti-coagulant (LA), anti-cardiolipin (aCL), and anti-β2 glycoprotein-I (β2GPI). To date, the therapeutic approach for thrombotic APS mainly centers on long-term anti-coagulation with a vitamin K antagonist (VKA). APS management may represent a challenge for the treating physicians. Patients with different aPL profiles need a tailored risk-stratified approach. Moreover, in patients with recurrent thrombotic events despite therapy with VKA, or in those with microvascular involvement, new therapeutic options are highly needed. In this review, we aim to elucidate recent findings about new aPL specifities, available risk scoring models, and novel therapeutic approaches in APS management.
Similar content being viewed by others
References
Miyakis S, Lockshin MD, Atsumi T et al (2006) International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 4(2):295–306
Mahler M, Norman GL, Meroni PL, Khamashta M (2012) Autoantibodies to domain 1 of beta 2 glycoprotein 1: a promising candidate biomarker for risk management in antiphospholipid syndrome. Autoimmun Rev 12(2):313–317
Radin M, Cecchi I, Roccatello D et al (2016) Prevalence and thrombotic risk assessment of anti-domain I beta 2 glycoprotein I antibodies: a systematic review and a meta-analysis. Lupus 25 (abstract supplement 1S)
Sciascia S, Khamashta MA, Bertolaccini ML (2014) New tests to detect antiphospholipid antibodies: antiprothrombin (aPT) and anti-phosphatidylserine/prothrombin (aPS/PT) antibodies. Curr Rheumatol Rep 16(5)
Ruffatti A, Del Ross T, Ciprian M et al (2011) Risk factors for a first thrombotic event in antiphospholipid antibody carriers: a prospective multicentre follow-up study. Ann Rheum Dis 70(6):1083–1086
Galli M, Luciani D, Bertolini G et al (2003) Lupus anticoagulants are stronger risk factors for thrombosis than anticardiolipin antibodies in the antiphospholipid syndrome: a systematic review of the literature. Blood 101(5):1827–1832
Groot PGDE, Lutters B, Derksen RHWM, Lisman T (2005) Lupus anticoagulants and the risk of a first episode of deep venous thrombosis. 1993–1997
Pengo V, Ruffatti A, Legnani C et al (2010) Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost 8(2):237–242
Otomo K et al (2012) Efficacy of the antiphospholipid score for the diagnosis of antiphospholipid syndrome and its predictive value for thrombotic events. Arthritis Rheum 64(2):504–512
Sciascia S, Sanna G, Murru V et al (2013) GAPSS: the global anti-phospholipid syndrome score. Rheumatology (Oxford) 52(8):1397–1403
Sciascia S, Baldovino S, Schreiber K et al (2016) Thrombotic risk assessment in antiphospholipid syndrome: the role of new antibody specificities and thrombin generation assay. Clin Mol Allergy 14(1):6
Sciascia S, Khamashta MA, D’Cruz DP (2014) Targeted therapy in antiphospholipid syndrome. Curr Opin Rheumatol 26(3):269–275
Rand JH, Wu X-X, Quinn AS et al (2010) Hydroxychloroquine protects the annexin A5 anticoagulant shield from disruption by antiphospholipid antibodies: evidence for a novel effect for an old antimalarial drug. Blood 115(11):2292–2299
Kaiser R, Cleveland CM, Criswell LA (2009) Risk and protective factors for thrombosis in systemic lupus erythematosus: results from a large, multi-ethnic cohort. Ann Rheum Dis 68(2):238–241
Cohen H, Hunt BJ, Efthymiou M, Arachchillage DR et al (2016) Rivaroxaban versus warfarin to treat patients with thrombotic antiphospholipid syndrome, with or without systemic lupus erythematosus (RAPS): a randomised, controlled, open-label, phase 2/3, non-inferiority trial. Lancet Haematol 3(9):e426–e436
Sciascia S, Giachino O, Roccatello D Prevention of thrombosis relapse in antiphospholipid syndrome patients refractory to conventional therapy using intravenous immunoglobulin. Clin Exp Rheumatol 30(3):409–413
Erre GL, Pardini S, Faedda R, Passiu G (2008) Effect of rituximab on clinical and laboratory features of antiphospholipid syndrome: a case report and a review of literature. Lupus 17(1):50–55
Sciascia S, Naretto C, Rossi D, Bazzan M, Roccatello D (2011) Treatment-induced downregulation of antiphospholipid antibodies: effect of rituximab alone on clinical and laboratory features of antiphospholipid syndrome. Lupus 20(10):1106–1108
Durcan L, Fu W, Petri M (2016) Hypocomplementemia associates with thrombosis in SLE patients with antiphospholipid antibodies. Ann Rheum Dis 75(Suppl2):126
Pengo V, Ruffatti A, Legnani C et al (2010) Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost 8(2):237–242
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Conflict of interest
The Authors declare that they have no conflict of interest.
Statement of human and animal rights
Not applicable.
Informed consent
Not applicable.
Funding
None declared.
Rights and permissions
About this article
Cite this article
Sciascia, S., Radin, M., Bazzan, M. et al. Novel diagnostic and therapeutic frontiers in thrombotic anti-phospholipid syndrome. Intern Emerg Med 12, 1–7 (2017). https://doi.org/10.1007/s11739-016-1596-2
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11739-016-1596-2