Recurrent Thrombosis Risk in Non-anticoagulated Antiphospholipid Syndrome Patients: A Prospective Case-Control Study from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”)

Cecile Yelnik¹, Zeynep Belce Erton², Elodie Drumez³, Dachi Cheildze², Danieli Castro Oliveira de Andrade⁴, Ann E Clarke⁵, Maria G. Tektonidou⁶, Vittorio Pengo⁷, Savino Sciascia⁸, Amaia Ugarte⁹, H Michael Belmont¹⁰, Mª Angeles Aguirre¹¹, Paul fortin¹², maria gerosa¹³, Flavio Victor Signorelli¹⁴, Tatsuya Atsumi¹⁵, Zhouli Zhang¹⁶, Hannah Cohen¹⁷, D. Ware Branch¹⁸, Denis Wahl¹⁹, Laura Andreoli²⁰, Esther Rodriguez Almaraz²¹, Michelle Petri²², Ricard Cervera²³, Yu Zuo²⁴, Bahar Artim-Esen²⁵, Guillermo Pons-Estel²⁶, Rohan Willis²⁷, Maria Laura Bertolaccini²⁸, Robert Roubey²⁹, Doruk Erkan² and on behalf of APS ACTION On Behalf Of APS ACTION², ¹Lille University, Lille, France, ²Hospital for Special Surgery, New York, NY, ³Université de Lille, Lille, France, ⁴Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de São Paulo, São Paulo, Brazil, ⁵University of Calgary, Division of Rheumatology, Cumming School of Medicine, Calgary, AB, Canada, ⁶National and Kapodistrian University of Athens, Athens, Greece, ⁷Padova University Hospital, Padova, Italy, ⁸University of Turin, Torino, Italy, ⁹BioCruces Bizkaia Health Research Institute, Barakaldo, Spain, ¹⁰NYU School of Medicine, New York, NY, ¹¹Maimonides Institute for Biomedical Research of Córdoba, Cordoba, Spain, ¹²CHU de Québec, Pavillon CHUL, Québec, Canada, ¹³University of Milan, Milano, Italy, ¹⁴Universidade do Estado do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁵Hokkaido University, Sapporo, Japan, ¹⁶Peking University First Hospital, Beijing, China, ¹⁷University College London, London, United Kingdom, ¹⁸University of Utah, Salt Lake City, UT, ¹⁹Université de Nancy, Nancy, France, ²⁰Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Brescia, Italy, ²¹Hospital Universitario 12 de Octubre, Madrid, Spain, ²²Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, ²³Hospital Clínic de Barcelona, Barcelona, Spain, ²⁴University of Michigan, Ann Arbor, MI, ²⁵Istanbul University, Istanbul, Turkey, ²⁶CREAR, Rosario, Argentina, ²⁷University of Texas Medical Branch, Galveston, TX, ²⁸King's College London, London, United Kingdom, ²⁹University of North Carolina, Chapel Hill, NC

Meeting: ACR Convergence 2022

Keywords: antiphospholipid syndrome, registry, risk assessment

Session Information

Date: Monday, November 14, 2022

Title: Abstracts: Antiphospholipid Syndrome

Session Type: Abstract Session

Session Time: 5:00PM-6:00PM

Background/Purpose: Long-term anticoagulation is the standard of care for antiphospholipid syndrome (APS) patients with macrovascular thrombosis. However, in daily practice, long-term anticoagulation may not be initiated, or eventually withdrawn for various reasons. This study aimed to describe the characteristics of non-anticoagulated thrombotic APS (TAPS) patients, and to analyze their risk of thrombosis recurrence in comparison to TAPS patients on long-term anticoagulation.

Methods: A web-based data capturing system is used to store patient demographics and relevant clinical and laboratory information. Patients are prospectively followed every 12±3 months and the occurrence of all new thrombotic events are recorded. Inclusion criteria of this prospective, observational, multicenter, case-control study of APS ACTION Registry analysis were: i) APS classification based on to the Updated Sapporo Criteria; ii) aged 18 or older; and iii) history of TAPS with at least one macrovascular thrombotic event. Cases were all TAPS patients not on long-term anticoagulation at the registry entry. For each case, control patients on long-term anticoagulation were included, matched based on gender, age (±5 years), type of previous thrombosis (arterial vs venous), date of registry entry (±1 year), and associated autoimmune disease. Statistical analyses included logistic mixed regression model for comparisons between groups, and a survival analysis using Kaplan-Meier curves and log-Rank test.

Results: As of May 2022, 662 TAPS patients were included in the registry. Ninety-one (14%) patients were not on anticoagulation at registry entry; 42/91 (46%) never received anticoagulation, while 49/91 (55%) had anticoagulation withdrawn before registry entry. Reasons for not receiving anticoagulation were known in 31 patients: provoked thrombosis with major transient risk factor (n=9), incidental finding (n=3), delayed APS diagnosis (n=4), thrombocytopenia (n=6), major hemorrhage (n=2), isolated retinal thrombosis (n=4), and patient’s decision (n=3). We identified 144 matched-controls for 86 of the 91 not-anticoagulated patients (cases). Demographical and clinical characteristics of two groups were similar at registry entry except cases had a higher frequency of chronic renal disease, lower mean BMI, and were more likely to receive antiplatelet therapy. During the prospective follow-up, six cases and four controls had at least one thrombosis; the relapse rates (cases vs controls) were 4% vs zero and 8.4% vs 3.2% at 2-year and 5-year follow-up, respectively. Despite a trend, the thrombosis-free survival did not differ significantly between cases and controls: hazard ratio 0.324 95%CI (0.09; 1.16) p=0.084.

Conclusion: Fourteen percent of thrombotic APS patients included in this international registry were not on long-term anticoagulation at recruitment, while the majority of these patients received an anti-platelet agent. Recurrent thrombosis risk in these non-anticoagulated patients only showed a trend towards being increased, compared to matched anticoagulated controls, supporting the urgent need for risk-stratified secondary thrombosis prevention trials in APS.

Disclosures: C. Yelnik, None; Z. Erton, None; E. Drumez, None; D. Cheildze, None; D. Castro Oliveira de Andrade, None; A. Clarke, AstraZeneca, Bristol Myers Squibb (BMS), GlaxoSmithKline (GSK); M. Tektonidou, None; V. Pengo, None; S. Sciascia, None; A. Ugarte, None; H. Belmont, None; M. Aguirre, None; P. fortin, None; m. gerosa, None; F. Signorelli, None; T. Atsumi, Alexion, Chugai Pharmaceutical, Daiichi Sankyo, Mitsubishi Tanabe Pharma, Otsuka Pharmaceutical, Pfizer, Teijin Pharma, AbbVie, Bristol-Myers Squibb (BMS), Eisai, Eli Lilly Japan, Kyowa Kirin, Novartis, Taiho Pharmaceutical, Takeda Pharmaceutical, UCB Pharma, AstraZeneca, Medical and Biological Laboratories, Boehringer-Ingelheim, Ono Pharmaceutical, Gilead Sciences, Inc., Astellas Pharma; Z. Zhang, None; H. Cohen, UCB Biopharmna, Springer, Roche; D. Branch, None; D. Wahl, None; L. Andreoli, GlaxoSmithKlein(GSK), Janssen, Novartis, UCB, Werfen; E. Rodriguez Almaraz, None; M. Petri, Exagen, AstraZeneca, Alexion, Amgen, AnaptysBio, Argenx, Aurinia, Biogen, Caribou Biosciences, CVS Health, EMD Serono, Eli Lilly, Emergent Biosolutions, GlaxoSmithKline (GSK), IQVIA, Janssen, Kira Pharmaceuticals, MedShr, Sanofi, SinoMab, Thermofisher, BPR Scientific Advisory Committee; R. Cervera, None; Y. Zuo, None; B. Artim-Esen, None; G. Pons-Estel, Janssen, GSK, Pfizer, Werfen/Inova, Novartis; R. Willis, None; M. Bertolaccini, UCB Biopharma SRL; R. Roubey, None; D. Erkan, None; o. On Behalf Of APS ACTION, None.

To cite this abstract in AMA style:

Yelnik C, Erton Z, Drumez E, Cheildze D, Castro Oliveira de Andrade D, Clarke A, Tektonidou M, Pengo V, Sciascia S, Ugarte A, Belmont H, Aguirre M, fortin P, gerosa m, Signorelli F, Atsumi T, Zhang Z, Cohen H, Branch D, Wahl D, Andreoli L, Rodriguez Almaraz E, Petri M, Cervera R, Zuo Y, Artim-Esen B, Pons-Estel G, Willis R, Bertolaccini M, Roubey R, Erkan D, On Behalf Of APS ACTION o. Recurrent Thrombosis Risk in Non-anticoagulated Antiphospholipid Syndrome Patients: A Prospective Case-Control Study from AntiPhospholipid Syndrome Alliance for Clinical Trials and InternatiOnal Networking (APS ACTION) Clinical Database and Repository (“Registry”) [abstract]. Arthritis Rheumatol. 2022; 74 (suppl 9). https://acrabstracts.org/abstract/recurrent-thrombosis-risk-in-non-anticoagulated-antiphospholipid-syndrome-patients-a-prospective-case-control-study-from-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-netwo/. Accessed .

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