0098: 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”
Roger Williams Medical Center Providence, RI, United States
Disclosure information not submitted.
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
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 .
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.