Predictors of Mortality in Antiphospholipid Antibody Positive Patients: Prospective Results from Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Clinical Database and Repository “Registry”

Yasaman Ahmadzadeh¹, Larry Magder², Zeynep Ozge Ozdemir³, Danieli Andrade⁴, Megan R.W. Barber⁵, Maria Tektonidou⁶, Savino Sciascia⁷, Vittorio Pengo⁸, Ariadna Anunciación-Llunell⁹, Guillermo Ruiz-Irastorza¹⁰, Mª Angeles Aguirre¹¹, Michael Belmont¹², Kello Nina¹³, Paul R. Fortin¹⁴, Denis WAHL¹⁵, Maria Gerosa¹⁶, Guilherme De Jesús¹⁷, Zhuoli Zhang¹⁸, Tatsuya Atsuma¹⁹, Maria Efthymiou²⁰, D. Ware Branch²¹, Angela Tincani²², Esther Rodriguez almaraz²³, Giulia Pazzola²⁴, Ricard Cervera²⁵, Bahar Artim Esen²⁶, Hui Shi²⁷, Jason Knight²⁸, Guillermo Pons-Estel²⁹, Rohan Willis³⁰, Ali Duarte-Garcia³¹, Maria Laura Bertolaccini³², Hannah Cohen³³, Michelle Petri³⁴, Doruk Erkan³⁵ and On Behalf Of APS ACTION³⁶, ¹Roger Williams Medical Center, Providence, RI, ²University of Maryland, Baltimore, MD, ³Hacettepe University Faculty of Medicine, Department of Internal Medicine, Ankara, Turkey, ⁴University of São Paulo, São Paulo, Brazil, ⁵University of Calgary, Calgary, AB, Canada, ⁶Joint Academic Rheumatology Program, First Department of Propedeutic and Internal Medicine, National and Kapodistrian University of Athens, Athens, Greece, ⁷University of Turin, Torino, Italy, ⁸Department of Cardiac-Thoracic-Vascular Sciences and Public Health University of Padova, Padova, Italy, ⁹VHIR Vall d'Hebron Institut de Investigación, Barcelona, Spain, ¹⁰Hospital Universitario Cruces, Barakaldo, Spain, ¹¹Reina Sofía University Hospital/ Rheumatology Department, Córdoba, Spain, ¹²New York University Langone Medical Center, New York, NY, ¹³Northwell Health, Brooklyn, NY, ¹⁴Centre ARThrite - CHU de Québec - Université Laval, Quebec City, QC, Canada, ¹⁵University of Lorraine, Nancy, France, ¹⁶University of Milan, Milano, Italy, ¹⁷Universidade do Estado do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁸Peking University First Hospital, Rheumatology and Immunology Department, Beijing, China, ¹⁹Hokkaido University, Department of Rheumatology, Endocrinology and Nephrology, Sapporo, Japan, ²⁰University College London, London, United Kingdom, ²¹University of Utah, Salt Lake City, UT, ²²Rheumatology and Clinical Immunology, ASST Spedali Civili of Brescia, University of Brescia, Gussago, Italy, ²³Hospital Universitario 12 de Octubre, Madrid, Spain, ²⁴Azienda Ospedaliera Santa Maria Nuova di Reggio Emilia, Reggio Emilia, Italy, ²⁵Hospital Clínic de Barcelona, Barcelona, Spain, ²⁶Istanbul University, Istanbul Faculty of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²⁷Rijin Hospital, Shanghai, China, ²⁸University of Michigan, Ann Arbor, MI, ²⁹CREAR, Rosario, Argentina, ³⁰University of Texas Medical Branch, Galveston, TX, ³¹Mayo Clinic, Rochester, MN, ³²King's College London, London, United Kingdom, ³³Haemostasis Research Unit, Department of Hematology, University College London, London, United Kingdom, ³⁴Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Timonium, MD, ³⁵Hospital for Special Surgery, New York, NY, ³⁶on behalf of APS ACTION, New York, NY

Meeting: ACR Convergence 2023

Keywords: antiphospholipid syndrome, autoimmune diseases, Mortality

Session Information

Date: Sunday, November 12, 2023

Title: (0096–0116) Antiphospholipid Syndrome Poster

Session Type: Poster Session A

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

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. Our objective was to determine the mortality rate as well as the causes and predictors of mortality in aPL-positive patients with/without APS classification.

Methods: A web-based data-capturing system is used to store patient demographics, history, and medications. The inclusion criteria are positive aPL according to the Updated Sapporo Classification Criteria tested within one year prior to enrollment. Patients are followed every 12 ± 3m with clinical data and blood collection. In this prospective analysis, firstly we analyzed descriptively the causes of death (based on investigators’ reports) for patients reported as “deceased” during the follow-up. Secondly, we compared the clinical and laboratory characteristics of deceased versus non-deceased patients using the adjusted Cox proportional hazards model and calculated the survival probability by Kaplan-Meier Model based on different age groups.

Results: As of May 2023, of 1,174 patients recruited, 215 (18%) were excluded due to incomplete follow-up data. Of the remaining 963 (mean age at registry entry: 53.0 ± 13.3, female: 723 [75%], and White: 657 [68%]), 43 (5%) were reported as deceased after a median follow-up of 5.3 years (interquartile range 2.4 to 7.9). The main causes of death (not mutually exclusive) were: infection (15, 35%), thrombosis (9, 21%), and malignancies (8, 19%). Based on the univariate analysis, history of arterial thrombosis or catastrophic APS (CAPS), selected baseline cardiovascular disease (CVD) risk factors, baseline active thrombocytopenia, and concomitant systemic autoimmune disease (SAID) were significantly more common in deceased patients, compared to non-deceased (data not shown). Based on the Cox proportional hazards model adjusted for age, arterial thrombosis, CAPS, CVD risk factors combined, and SAID were significantly and independently associated with mortality (Tables 1 and 2). Estimated 5-year survival probabilities starting from the registry entry (by age groups) were 0.98 (95% CI 0.92-0.99), 0.98 (0.95-0.99), 0.96 (0.93-0.98), and 0.86 (0.77-0.92) for ages < 30 (n:119), 30-44 (n: 362), 45-59 (n: 340), and >60 (n: 142), respectively (Figure 1).

Conclusion: Based on analysis of the largest multi-center international prospective cohort of persistently aPL-positive patients, the mortality rate was 5% after a median follow-up of five years. The estimated 5-year survival probability decreased with age; lowest (0.86) for patients over 60 years old at registry entry. History of arterial thrombosis, catastrophic APS, CVD risk factors, and concomitant systemic autoimmune diseases independently predicted future mortality
.

Disclosures: Y. Ahmadzadeh: None; L. Magder: None; Z. Ozdemir: None; D. Andrade: None; M. Barber: AbbVie/Abbott, 2, AstraZeneca, 2, GlaxoSmithKlein(GSK), 2, Janssen, 2, Sanofi-Genzyme, 2; M. Tektonidou: None; S. Sciascia: None; V. Pengo: Werfen group, Milan, Italy, 6; A. Anunciación-Llunell: None; G. Ruiz-Irastorza: None; M. Aguirre: None; M. Belmont: None; K. Nina: None; P. Fortin: AbbVie, 1, AstraZeneca, 1, 6, GlaxoSmithKlein(GSK), 1, 6, Roche-Genentech, 1; D. WAHL: None; M. Gerosa: None; G. De Jesús: GlaxoSmithKlein(GSK), 5, UCB, 1; Z. Zhang: None; T. Atsuma: AbbVie, 5, 6, Alexion, 5, 6, Astellas, 5, 6, Boehringer-Ingelheim, 2, Bristol-Myers Squibb, 6, Chugai, 5, 6, Daiichi Sankyo, 5, 6, Eisai, 5, 6, Eli Lilly, 5, 6, Gilead, 5, 6, GSK, 2, 5, Merck Sharp & Dohme, 2, 6, Mitsubishi Tanabe Pharma, 5, 6, Otsuka, 5, 6, Pfizer, 5, 6, Sanofi/Genzyme, 2, 6, Takeda, 5, 6, UCB, 5, 6; M. Efthymiou: Alexion, 1, Immune Tolerance Network (ITN), 1; D. Branch: UCB Pharmaceuticals, 5; A. Tincani: Galapagos, 6, GSK, 6, UCB, 6; E. Rodriguez almaraz: None; G. Pazzola: None; R. Cervera: None; B. Artim Esen: None; H. Shi: None; J. Knight: Jazz Pharmaceuticals, 2; G. Pons-Estel: GlaxoSmithKlein(GSK), 1, 5, 6, Janssen, 1, 5, 6, Novartis, 1, 6, Pfizer, 5, 6, Werfen/Inova, 5, 6; R. Willis: None; A. Duarte-Garcia: None; M. Bertolaccini: None; H. Cohen: None; M. Petri: Alexion, 1, Amgen, 1, AnaptysBio, 1, Annexon Bio, 1, Argenx, 1, Arhros-Focus Med/Ed, 6, AstraZeneca, 1, 5, Aurinia, 1, 5, 6, Axdev, 1, Biogen, 1, Boxer Capital, 2, Cabaletto Bio, 2, Caribou Biosciences, 2, CVS Health, 1, Eli Lilly, 1, 5, Emergent Biosolutions, 1, Exagen, 5, Exo Therapeutics, 2, Gilead Biosciences, 2, GlaxoSmithKlein(GSK), 1, 5, 6, Horizon Therapeutics, 2, Idorsia Pharmaceuticals, 2, IQVIA, 1, Janssen, 1, 5, Kira Pharmaceuticals, 2, MedShr, 6, Merck/EMD Serono, 1, Momenta Pharmaceuticals, 2, Nexstone Immunology, 2, Nimbus Lakshmi, 2, Proviant, 2, Sanofi, 2, Sinomab Biosciences, 2, Thermofisher, 5, UCB, 2; D. Erkan: Abbvie, 1, ACR/EULAR, 5, APS ACTION, 12, Executive Committee Co-chair, Argenx, 1, Aurinia, 6, Chugai, 1, Exagen, 5, GSK, 5, 6, NIH-NIAID, 5, Up-To-Date, 9; O. Of APS ACTION: None.

To cite this abstract in AMA style:

Ahmadzadeh Y, Magder L, Ozdemir Z, Andrade D, Barber M, Tektonidou M, Sciascia S, Pengo V, Anunciación-Llunell A, Ruiz-Irastorza G, Aguirre M, Belmont M, Nina K, Fortin P, WAHL D, Gerosa M, De Jesús G, Zhang Z, Atsuma T, Efthymiou M, Branch D, Tincani A, Rodriguez almaraz E, Pazzola G, Cervera R, Artim Esen B, Shi H, Knight J, Pons-Estel G, Willis R, Duarte-Garcia A, Bertolaccini M, Cohen H, Petri M, Erkan D, Of APS ACTION O. Predictors of Mortality in Antiphospholipid Antibody Positive Patients: Prospective Results from Antiphospholipid Syndrome Alliance for Clinical Trials and International Networking (APS ACTION) Clinical Database and Repository “Registry” [abstract]. Arthritis Rheumatol. 2023; 75 (suppl 9). https://acrabstracts.org/abstract/predictors-of-mortality-in-antiphospholipid-antibody-positive-patients-prospective-results-from-antiphospholipid-syndrome-alliance-for-clinical-trials-and-international-networking-aps-action-clinic/. Accessed .

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