First and Recurrent Thrombosis Risk After 3842 Patient-Years of Follow-Up: Prospective Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (

Yasaman Ahmadzadeh¹, Danieli Andrade², Maria Tektonidou³, Vittorio Pengo⁴, Savino Sciascia⁵, Amaia Ugarte⁶, H. Michael Belmont⁷, Maria Gerosa⁸, Paul R Fortin⁹, Maria Angeles Aguirre¹⁰, Lanlan Ji¹¹, Tatsuya Atsumi¹², Hannah Cohen¹³, Guilherme Ramires de Jesus¹⁴, D. Ware Branch¹⁵, Angela Tincani¹⁶, Nina Kello¹⁷, Michelle Petri¹⁸, Esther Rodriguez-Almaraz¹⁹, Roberto Ríos-Garcés²⁰, Yu Zuo²¹, Bahar Artim-Esen²², Rohan Willis²³, Maria Laura Bertolaccini²⁴, Robert Roubey²⁵, Doruk Erkan¹ and on Behalf of APS ACTION¹, ¹Hospital for Special Surgery, New York, NY, ²Hospital das Clinicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil, ³FORZAFORTE HELLAS LTD, Athens, Greece, ⁴Padova University Hospital, Padova, Italy, ⁵University of Turin, Turin, Italy, ⁶Hospital Universitario Cruces, Barakaldo, Spain, ⁷NYU School of Medicine, New York, NY, ⁸University of Milan, Milan, Italy, ⁹CHU de Quebec - Universite Laval, Québec City, QC, Canada, ¹⁰IMIBIC/Reina Sofia Hospital/University of Córdoba, Córdoba, Spain, ¹¹Peking University First Hospital, Beijing, China (People's Republic), ¹²Hokkaido University, Sapporo, Japan, ¹³Department of Haematology, University College London Hospitals NHS Foundation Trust, London, United Kingdom, ¹⁴Universidade do Estado do Rio de Janeiro, Rio de Janeiro, Brazil, ¹⁵University of Utah, Salt Lake City, UT, ¹⁶ASST Spedali Civili-University of Brescia, Gussago, Italy, ¹⁷Zucker School of Medicine at Hofstra/Northwell Health, Manhasset, NY, ¹⁸Johns Hopkins University School of Medicine, Baltimore, MD, ¹⁹Hospital Universitario 12 de Octubre, Madrid, Spain, ²⁰Hospital Clínic de Barcelona, Barcelona, Spain, ²¹University of Michigan, Ann Arbor, MI, ²²Istanbul University School of Medicine, İstanbul, Turkey, ²³University of Texas Medical Branch, Galveston, TX, ²⁴King's College London, London, United Kingdom, ²⁵University of North Carolina, Chapel Hill, NC

Meeting: ACR Convergence 2021

Keywords: antiphospholipid syndrome, Thrombosis

Session Information

Date: Saturday, November 6, 2021

Title: Antiphospholipid Syndrome Poster (0069–0083)

Session Type: Poster Session A

Session Time: 8:30AM-10:30AM

Background/Purpose: APS ACTION “Registry” was created to study the natural course of disease over 10 years in persistently antiphospholipid antibody (aPL)-positive patients with/without other systemic autoimmune diseases (SAIDx). The objective of this study was to update the incident first and recurrent thrombosis risk in persistently aPL-positive registry patients, previously reported in 2018 as 1.53% and 2.75%, respectively (Arthritis Rheumatol.2018;70[suppl 10]).

Methods: A web-based data capturing system is used to store patient demographics, history, and medications. The inclusion criteria are positive aPL according to Updated Sapporo Classification Criteria tested within one year prior to the enrollment. Patients are followed every 12±3m with clinical data and blood collection; they also receive counseling on cardiovascular disease (CVD) and venous thromboembolism (VTE) prevention at each visit. In this prospective analysis, based on patients who completed 1-8-year follow-up visits, we report the incident thrombosis risk in persistently aPL-positive patients without and with a history of thrombosis. We also compare the demographic and clinical characteristics of patients with and without new thrombosis.

Results: As of March 2021, 886 patients were included: aPL/APS without SAIDx: 546 (aPL without APS classification: 101; thrombotic APS [TAPS]: 320; obstetric APS [OAPS]: 59; and TAPS+OAPS: 66]; and aPL/APS associated with SAIDx: 340 [aPL without APS: 91; TAPS: 178; OAPS: 21; and TAPS+OAPS: 50]). Of 886 patients, 705, 605, 527, 456, 401, 328, 200 and 49 completed 1, 2, 3, 4, 5, 6, 7, and 8-year follow-up, respectively. Mean follow-up was 4.67 years (1270 patient-years [pt-y]) and 4.19 years (2572 pt-y) for those without and with a history of thrombosis, respectively. Based on 13 initial events in 13 patients, and 67 recurrent events in 54 patients (Tables 1 and 2), the incident thrombosis risk was 1.02 and 2.09 per 100 pt-y in patients without and with a history of thrombosis, respectively. Demographics, concomitant lupus, aPL-profile, medications, and non-aPL thrombosis risk factors were not different between aPL-positive patients with (n=13) or without (n=259) first thrombosis, and between APS patients with (n=54) or without (n=560) recurrent thrombosis except APS patients with recurrence were younger and more likely to have triple aPL positivity compared to those without recurrence (42.1 ± 14.1 vs 46.6 ± 13.3, p 0.02, and 31% vs 50%, p 0.006).

Conclusion: Based on approximately 4000 patient-years of follow-up, the incident thrombosis risk in persistently aPL-positive patients remains relatively low (1.02 and 2.09 per 100 pt-y in patients without and with a history of thrombosis, respectively). Lupus anticoagulant and/or triple aPL-positivity, sub-therapeutic international normalized ratios, and additional CVD and VTE risk factors were relatively common at the time of the new events. Future Cox proportional hazards analysis will help us better define the risk and protective factors for thrombosis in persistently aPL-positive patients.

Disclosures: Y. Ahmadzadeh, None; D. Andrade, None; M. Tektonidou, None; V. Pengo, None; S. Sciascia, None; A. Ugarte, None; H. Belmont, Alexion, 6; M. Gerosa, None; P. Fortin, Lilly, 1, AbbVie, 1, AstraZeneca, 1; M. Aguirre, None; L. Ji, None; T. Atsumi, Takeda Pharmaceutical CO., Ltd., 6, Astellas Pharma Inc., 5, 6, Mitsubishi Tanabe Pharma Co., 5, 6, Chugai Pharmaceutical Co., Ltd., 5, 6, Daichii Sankyo Co. Ltd., 5, 6, Pfizer Inc., 2, 5, 6, Alexion Inc., 6, TEIJIN PHARMA LIMITED., 5, 6, Novartis Pharma K.K., 2, 5, 6, Eli Lilly Japan K.K., 5, 6, Kyowa Kirin Co., Ltd., 5, 6, AbbVie Inc., 2, 5, 6, NIPPON SHINYAKU CO.,LTD., 5, TAIHO PHARMACEUTICAL CO.,LTD., 5, Nippon Boehringer Ingelheim Co.,Ltd., 5, 6, Amgen Inc., 5, 6, UCB Japan Co. Ltd., 5, 6, Astra Zeneca plc, 2, 6, ONO Pharmaceutical Co., Ltd., 2, 5, Byaer Yakuhin, Ltd., 5; H. Cohen, Bayer Healthcare, 5, 6, 12, Support to attend scientific meetings; Honoraria for lectures at symposia paid to University College London Hospitals Charity, UCB Biopharma, 2, 12, Consultancy fees paid to University College London Hospitals Charity; G. de Jesus, None; D. Branch, UCB Pharmaceuticals, 1, 5; A. Tincani, Novartis, 2, UCB, 2, Janssen, 2, Gsk, 6; N. Kello, None; M. Petri, Alexion, 1, Amgen, 1, Astrazeneca, 1, 5, Aurinia, 5, 6, Eli Lilly, 5, Emergent Biosolutions, 1, Exagen, 5, Gilead Biosciences, 2, GSK, 1, 5, IQVIA, 1, Idorsia Pharmaceuticals, 2, Janssen, 1, 5, Merck EMD Serono, 1, Momenta Pharmaceuticals, 2, PPD Development, 1, Sanofi, 2, Thermofisher, 5, UCB Pharmaceuticals, 2; E. Rodriguez-Almaraz, None; R. Ríos-Garcés, GlaxoSmithKline, 12, Registration to congresses; Y. Zuo, None; B. Artim-Esen, None; R. Willis, Louisville APL Diagnostic Inc, 2; M. Bertolaccini, None; R. Roubey, None; D. Erkan, ACR/EULAR, 5, LCTC, 5, NIH/NIAID, 5, GSK, 5, 6, Exagen, 5, Alexion, 2, UCB, 2, UpToDate, 9, APS ACTION, 4; o. APS ACTION, None.

To cite this abstract in AMA style:

Ahmadzadeh Y, Andrade D, Tektonidou M, Pengo V, Sciascia S, Ugarte A, Belmont H, Gerosa M, Fortin P, Aguirre M, Ji L, Atsumi T, Cohen H, de Jesus G, Branch D, Tincani A, Kello N, Petri M, Rodriguez-Almaraz E, Ríos-Garcés R, Zuo Y, Artim-Esen B, Willis R, Bertolaccini M, Roubey R, Erkan D, APS ACTION o. First and Recurrent Thrombosis Risk After 3842 Patient-Years of Follow-Up: Prospective Results from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository ( [abstract]. Arthritis Rheumatol. 2021; 73 (suppl 9). https://acrabstracts.org/abstract/first-and-recurrent-thrombosis-risk-after-3842-patient-years-of-follow-up-prospective-results-from-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-networking-aps-action-cli/. Accessed .

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