The Clinical and Laboratory Characteristics of Antiphospholipid Antibody Positive Patients Included in the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ("Registry")

Ecem Sevim¹, Diane Zisa ², Danieli Andrade ³, Vittorio Pengo ⁴, Savino Sciascia ⁵, Maria Tektonidou ⁶, Amaia Ugarte ⁷, Maria Gerosa ⁸, H Michael Belmont ⁹, Rosario Lopez Pedrera ¹⁰, Lanlan Ji ¹¹, Paul Fortin ¹², Maria Efthymiou ¹³, Guilherme De Jesus ¹⁴, David Branch ¹⁵, Laura Andreoli ¹⁶, Michelle Petri ¹⁷, Ricard Cervera ¹⁸, Esther Rodriguez ¹⁹, Jason Knight ²⁰, Tatsuya Atsumi ²¹, Rohan Willis ²², Maria Laura Bertolaccini ²³, Hannah Cohen ¹³, Robert Roubey ²⁴, Doruk Erkan ²⁵, Medha Barbhaiya ² and on Behalf of APS ACTION ²⁶, ¹Hospital for Special Surgery, New York, NY, ²Hospital for Special Surgery, Weill Cornell Medicine, New York, NY, ³Hospital das Clinicas, Faculdade de Medicina, Universidade de São Paulo, Sao Paulo, Brazil, ⁴Azienda Ospedaliera of Padova, University of Padova, Padova, Italy, ⁵Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Torino, Italy, ⁶First Department of Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ⁷Hospital Universitario Cruces, University of the Basque Country Autoimmune Diseases Research Unit, Department of Internal M edicine, BioCruces Health, Biscay, Spain, ⁸Istituto Ortopedico Gaetano Pini, University of Milan, Milan, Italy, ⁹NYU Langone Health, New York, NY, ¹⁰Maimonides Institute for Biomedical Research of Cordoba, Cordoba, Spain, ¹¹Peking University First Hospital, Beijing, China (People's Republic), ¹²Division de Rhumatologie, Département de Médecine, CHU de Québec – Université Laval, Axe maladies infectieuses et inflammatoires, Centre de recherche du CHU de Québec – Université Laval, Canada, Quebec, QC, Canada, ¹³University College London, London, United Kingdom, ¹⁴Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁵University of Utah Health Sciences Center, Salt Lake City, UT, ¹⁶Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of Brescia, Brescia, Italy, Brescia, Italy, ¹⁷Johns Hopkins University School of Medicine, Baltimore, MD, ¹⁸Department of Autoimmune Diseases, Hospital Clínic, Barcelona, Barcelona, Catalonia, Spain, ¹⁹Hospital Universitario 12 de Octubre, Madrid, Spain, ²⁰Division of Rheumatology, Department of Internal Medicine, Michigan Medicine, Ann Arbor, MI, ²¹Hokkaido University, Sapporo, Japan, ²²Antiphospholipid Standardization Laboratory, University of Texas Medical Branch, Galveston, TX, ²³King's College London, London, United Kingdom, ²⁴The University of North Carolina at Chapel Hill, Chapel Hill, NC, ²⁵Hospital for Special Surgery, New York City, ²⁶APS ACTION, New York, NY

Meeting: 2019 ACR/ARP Annual Meeting

Keywords: Antiphospholipid antibodies, antiphospholipid syndrome, thrombosis and international

Session Information

Date: Sunday, November 10, 2019

Title: Antiphospholipid Syndrome Poster

Session Type: Poster Session (Sunday)

Session Time: 9:00AM-11:00AM

Background/Purpose: APS ACTION “Registry” was created to study the long-term natural history and outcomes of persistently antiphospholipid antibody (aPL)-positive patients with and without other systemic autoimmune diseases (SAID). Our primary objective was to describe baseline demographic, clinical, and laboratory characteristics of patients enrolled since 2010.

Methods: A web-based data capture system is used to store patient demographics and aPL-related medical history. APS ACTION Registry includes adults aged 18 to 60 years with positive aPL, based on the Updated Sapporo APS Classification Criteria, tested on two occasions at least twelve weeks apart within one year prior to enrollment. Patients are followed every 12±3m with clinical data and blood collection. For this retrospective analysis, we descriptively evaluated the sociodemographic and clinical characteristics of patients overall and in categories by disease manifestation (aPL positive without APS, thrombotic APS, and obstetric APS). We also assessed the clinical characteristics of aPL-positive patients who were tested for all three aPL (lupus anticoagulant [LA] test, anticardiolipin antibody [aCL], and anti-β₂-Glycoprotein-I [aβ₂GPI]) by subgroups based on aPL profiles (LA only, single, double, and triple positivity). Positivity for aCL IgG/M/A and aβ₂GPI IgG/M/A was defined as a titer of ≥40 GPL/MPL/APL units and the highest titer among all test results was taken into consideration during analysis.

Results: As of March 2019, 804 patients were enrolled from 26 centers (mean age: 45 ± 13y; female: 74%; white 68%; and other SAID: 36%). Overall, 172 (21%) were aPL-positive without APS, 453 (56%) thrombotic APS; 73 (9%) purely obstetric APS; and 106 (13%) both thrombotic and obstetric APS. Among thrombotic APS patients, there were slightly higher rates of venous vs arterial thrombosis (62% vs 49%). Slightly higher rates of at least one non-criteria manifestation were observed in APS versus aPL only patients (58% vs 49%). All three aPL were tested in 668 (83%) patients, of whom 278 (42%) were triple positive. While similar frequencies of overall vascular thrombosis, pregnancy morbidity, and non-criteria manifestation were seen across single, double, and triple positivity subgroups, the lowest frequencies were observed in the single aPL positivity (excluding lupus anticoagulant [LA] only) subgroup (Table).

Conclusion: One-fifth of APS ACTION patients do not fulfill clinical APS classification criteria; 70% have vascular events; one-fifth have obstetric morbidity; and more than half have at least one non-criteria manifestation. Within single aPL-positivity, LA positivity appears to be an important contributor to aPL-related clinical features. Future prospective analyses, using standardized core laboratory aPL tests, will help better clarify aPL risk profiles.

Disclosure: E. Sevim, None; D. Zisa, None; D. Andrade, None; V. Pengo, None; S. Sciascia, None; M. Tektonidou, None; A. Ugarte, None; M. Gerosa, None; H. Belmont, None; R. Lopez Pedrera, None; L. Ji, None; P. Fortin, None; M. Efthymiou, None; G. De Jesus, None; D. Branch, None; L. Andreoli, None; M. Petri, Eli Lilly and Company, 5, Exagen, 2, 5; R. Cervera, None; E. Rodriguez, None; J. Knight, None; T. Atsumi, AbbVie, 5, 8, Abbvie, 5, 8, Asahi Kasei Pharma Corporation, 8, Astellas Pharma, 8, 9, Astellas Pharma Inc, 8, AstraZeneca, 5, AstraZeneca plc, 5, 8, Bayer Yakuhin, 8, Bayer Yakuhin, Ltd., 8, Bristol-Myers Squibb, 8, 9, Chugai Pharmaceutical Co Ltd, 8, Chugai Pharmaceutical Co., 8, 9, Daiichi Sankyo, 8, 9, Daiichi Sankyo Co Ltd, 8, Eisai Co., Ltd, 8, Eli Lilly and Company, 8, 9, Eli Lilly Japan KK, 8, Elsai Co Ltd, 8, Gilead Sciences, 8, Gilead Sciences, Inc., 8, MEDICAL & BIOLOGICAL LABORATORIES CO., 5, Medical and Biological Laboratories Co Ltd, 5, Mitsubishi Tanabe Pharma, 8, 9, Nippon Shinyaku Co., 8, Novartis, 5, Novartis Pharma KK, 5, Ono Pharmaceutical, 5, ONO Pharmaceutical Co Ltd, 5, Otsuka Pharmaceutical, 8, Pfizer, 5, 9, Pfizer Inc, 5, 8, Sanofi, 9, Takeda Pharmaceutical Company, 8, Takeda Pharmaceuticals, 8; R. Willis, None; M. Bertolaccini, None; H. Cohen, None; R. Roubey, None; D. Erkan, None; M. Barbhaiya, None; o. APS ACTION, None.

To cite this abstract in AMA style:

Sevim E, Zisa D, Andrade D, Pengo V, Sciascia S, Tektonidou M, Ugarte A, Gerosa M, Belmont H, Lopez Pedrera R, Ji L, Fortin P, Efthymiou M, De Jesus G, Branch D, Andreoli L, Petri M, Cervera R, Rodriguez E, Knight J, Atsumi T, Willis R, Bertolaccini M, Cohen H, Roubey R, Erkan D, Barbhaiya M, APS ACTION o. The Clinical and Laboratory Characteristics of Antiphospholipid Antibody Positive Patients Included in the AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”) [abstract]. Arthritis Rheumatol. 2019; 71 (suppl 10). https://acrabstracts.org/abstract/the-clinical-and-laboratory-characteristics-of-antiphospholipid-antibody-positive-patients-included-in-the-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-networking-aps-acti/. Accessed .

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