Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by clinical symptoms of vascular thrombosis and/or pregnancy morbidity in the presence of autoimmune antiphospholipid antibodies (aPL). Current laboratory APS criteria include the presence of at least one of the three relevant aPL: lupus anticoagulant, anticardiolipin antibodies and anti-β2 glycoprotein I antibodies. Therefore, patients could have a single aPL pattern or combinations of aPL. Evidence arising from clinical experience indicates that patients having the highest aPL titer and simultaneous aPL detected by different tests have a worse prognosis and a higher probability of recurrence of the APS clinical features. In recent years, an emerging role of multiple aPL positivity in the identification of high-risk patients with aPL/APS is evident. This paper will review the current knowledge on the clinical relevance of having single or multiple aPL positivity.

The emerging role of multiple antiphospholipid antibodies positivity in patients with antiphospholipid syndrome

Antiphospholipid syndrome (APS) is a clinical condition characterized by recurrent thrombosis and obstetric-related adverse events associated with the persistent presence of antiphospholipid antibodies (aPL). The aPL family is a heterogeneous group of autoantibodies with different antigenic specificities. The dominant antigenic targets for aPL in APS are phospholipid-binding plasma proteins such as β2glycoprotein I (β2GPI) and prothrombin. In the laboratory, aPLs can be detected by a diverse range of tests. There is not a unique or gold-standard test for the diagnosis of APS. The aPL tests can be broadly categorized into two groups: solid-phase assays such as anticardiolipin antibodies, anti-β2GPI antibodies, antiprothrombin antibodies, or phosphatidylserine-dependent antiprothrombin antibodies, and functional assays characterized by their ability to prolong phospholipid-dependent coagulation tests, known as lupus anticoagulant. In this chapter, we detail the currently available laboratory assays for aPL detection, and discuss the value of each aPL as laboratory marker for APS.

Laboratory Markers With Clinical Significance in the Antiphospholipid Syndrome

Background Phosphatidylserine-dependent antiprothrombin antibodies (aPS/PT) are strongly correlated with lupus anticoagulant. A Task Force of scientists at the International Congress on Antiphospholipid Antibodies (APLA 2010) accepted that aPS/PT would potentially contribute for a better identification of antiphospholipid syndrome (APS). Accordingly,we carried out an Initial and a Validation retrospective, cross-sectional multi-centre study on aPS/PT. Objectives To ascertain the value of aPS/PT for APS diagnosis. Methods We conducted an initial study involving 8 centres from 7 countries.Demographics and clinical/laboratory data were retrospectively collected.A sample from the subjects was prepared at each institution.Specimens were blinded and determination of IgG aPS/PT performed at Inova Diagnostics, Inc.USA (Inova) using ELISA kits from two manufacturers:Medical and Biological Laboratories Co Ltd, Japan (MBL) and Inova.After completing the initial study, a Validation study with a new sample of subjects (5 different centres from 5 countries) was carried out using the same methodology. Results The Initial study comprised 247 subjects.Statistically significant correlation in the IgG aPS/PT values was obtained with both ELISA kits (r=0.827, p Conclusions The performance of IgG aPS/PT using Inova and MBL ELISA kits is reliable. IgG aPS/PT detection is an easily performed laboratory parameter that may help in the diagnosis of APS. Acknowledgements The authors want to acknowledge the contributions of the late Prof. Silvia Pierangeli, University of Texas Medical Branch, Galveston, TX, USA Disclosure of Interest O. Amengual: None declared, R. Forastiero: None declared, M. Sugiura-Ogasawara: None declared, K. Otomo: None declared, O. Kenji: None declared, C. Favas: None declared, J. Delgado Alves: None declared, P. Žigon: None declared, A. Ambrožic: None declared, M. Tomsic: None declared, I. Ruiz Arruza: None declared, G. Ruiz Irastorza: None declared, M. Bertolaccini: None declared, G. Norman Employee of: Inova Diagnostics, Inc, San Diego, CA USA, Z. Shums: None declared, A. Jiro Employee of: Medical and Biological Laboratories, Co. Ltd, A. Murashima: None declared, A. Tebo: None declared, M. Gerosa: None declared, P. Meroni: None declared, I. Rodriguez-Pintό: None declared, R. Cervera: None declared, J. Swadzba: None declared, J. Musial: None declared, T. Atsumi Grant/research support from: Chugai Pharmaceutical Co., Ltd., Eisai Co., Ltd., Bristol-Myers Squibb Co., Astellas Pharma Inc., Daiichi Sankyo Co., Ltd. and Mitsubishi Tanabe Pharma Co., Speakers bureau: Astellas Pharma Inc. and Mitsubishi Tanabe Pharma Co.

https://ard.bmj.com/content/annrheumdis/74/Suppl_2/155.2.full.pdf

OP0223 Significance of IGG Phosphatidylserine-Dependent Antiprothrombin Antibody Testing for the Diagnosis of Antiphospholipid Syndrome: Results from the Initial and Validation International Multi-Centre Studies

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OP0223 Significance of IGG Phosphatidylserine-Dependent Antiprothrombin Antibody Testing for the Diagnosis of Antiphospholipid Syndrome: Results from the Initial and Validation International Multi-Centre Studies