ACR Meeting Abstracts

ACR Meeting Abstracts

Abstract Number: 0842

The Incident Risk and Predictors of Systemic Autoimmune Rheumatic Disease (SARD) Development in Persistently Antiphospholipid Antibody Positive Patients Without SARDs: Prospective Results from the APS ACTION Clinical Database and Repository (“Registry”)

Reyhan Kose Cobanoglu1, Rohan Willis2, Diana Paredes-Ruiz3, Maria G Tektonidou4, Vittorio Pengo5, Savino Sciascia6, Cecilia Nalli7, Flavio Victor Signorelli8, Paul Fortin9, Maria Efthymiou10, H Michael Belmont11, Michelle Petri12, Ricard Cervera13, Megan Barber14, TATSUYA ATSUMI15, Chary Lopez-Pedrera16, Jason Knight17, David Branch18, Nina Kello19, Lanlan Ji20, Esther Rodriguez-Almaraz21, Bahar Artim Esen22, Jose Pardos Gea23, Rosana Quintana24, Giulia Pazzola25, Hui Shi26, Ali Duarte-Garcia27, Pierluigi Meroni28, Robert Roubey29, Maria Laura Bertolaccini30, Hannah Cohen31, Danieli Andrade32 and Doruk Erkan33, and on behalf of APS ACTION, 1Aydin Adnan Menderes University School of Medicine, Hospital for Special Surgery, NY, USA, Aydin, Turkey, 2University of Texas Medical Branch, Galveston, TX, 3Biocruces Bizkaia Health Research Institute, Bizkaia, Spain, 4National and Kapodistrian University of Athens, Athens, Greece, 5Thrombosis Research Laboratory, Department of Cardio-Thoracic-Vascular Sciences and Public Health, University of Padova, Padova, Italy, 6University of Turin, Torino, Turin, Italy, 7ASST SPEDALI CIVILI DI BRESCIA, Brescia, Italy, 8Universidade do Estado do Rio de Janeiro, Rio De Janeiro, Brazil, 9Centre ARThrite - CHU de Québec - Université Laval, Quebec, QC, Canada, 10University College London, London, United Kingdom, 11NYU School of Medicine, New York, NY, 12Johns Hopkins University School of Medicine, Timonium, MD, 13Hospital Clinic de Barcelona, Barcelona, Spain, 14Division of Rheumatology, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, 15Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan, 16IMIBIC/Reina Sofia Hospital/University of Cordoba, Cordoba, Andalucia, Spain, 17University of Michigan, Ann Arbor, MI, 18University of Utah and Intermountain Healthcare, Salt Lake City, UT, 19Northwell Health, Brooklyn, NY, 20Peking University First Hospital, Peking, China (People's Republic), 21Hospital Universitario 12 de Octubre, Madrid, Spain, 22Istanbul University, Istanbul Faculty of Medicine, Division of Rheumatology, Istanbul, Turkey, 23Vall d'Hebron University Hospital, Barcelona, Spain, 24Centro Regional de Enfermedades Autoinmunes y Reumáticas (GO-CREAR), Rosario, Argentina, 25Rheumatology Unit, Azienda USL IRCCS di Reggio Emilia, Reggio Emilia, Italy, 26Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, Shanghai, China (People's Republic), 27Mayo Clinic, Rochester, MN, 28IRCCS Istituto Auxologico Italiano 100%, Cusano Milanino, Milan, Milan, Italy, 29Division of Rheumatology, Allergy, and Immunology, University of North Carolina, Chapel Hill, NC, 30King's College London, London, United Kingdom, 31University College London Hospitals NHS Foundation Trust, London, United Kingdom, 32University of São Paulo, São Paulo, SP, Brazil, 33Hospital for Special Surgery, New York, NY

Meeting: ACR Convergence 2024

Keywords: , ,

Session Information

Date: Saturday, November 16, 2024

Title: Abstracts: Antiphospholipid Syndrome

Session Type: Abstract Session

Session Time: 3:00PM-4:30PM

Background/Purpose: APS ACTION “Registry” was created to study the natural course of disease in persistently antiphospholipid antibody (aPL)-positive patients with/without other systemic autoimmune rheumatic diseases (SARDs). Our objective was to determine the risk and predictors of developing additional SARDs during the prospective follow-up of primary aPL-positive patients with/without antiphospholipid syndrome (APS) classification (primary aPL/APS).

Methods: A web-based data capturing system is used to store patient demographics, aPL-related history, 1997 Updated ACR Lupus Classification Criteria, and medications. The inclusion criteria for the registry are positive aPL according to the Revised Sapporo Classification Criteria, tested within one year prior to enrollment. Patients are followed every 12±3 months with clinical data and blood collection. In this prospective analysis, based on primary aPL/APS patients who had at least one-year follow-up visits, we report the incident risk and predictors of new SARDs using Cox proportional hazards model.

Results: As of March 2024, of 1,209 patients recruited, 487 (40%) were excluded due to concomitant SARDs at baseline, and 108 (9%) due to incomplete follow-up data. Of the remaining 614 patients (median age at registry entry: 46.9 [interquartile range 17.9-78.7], female: 442 [72%] and white: 434/570 [76%]), 28 (5%) developed a new SARD (lupus 17, other 11) during the median follow-up of six years (interquartile range 1.9 to 8.7) (longer in patients with new SARDs [Table 1]). The incident SARD risk was 0.76 per 100 patient-years based on 3,684 patient year follow-up. Based on the univariate Cox regression analysis, Asian race, brain white matter hyper-intensities, arthritis, lupus-like disease (3 of 11 ACR 1997 lupus classification criteria), anti-ribonuclear antibody (anti-RNP) positivity, low C4 levels, and rituximab (RTX) use were significantly associated with a new SARD development (Table 2). Based on the multivariate Cox regression analyses adjusted for confounders in two different models (clinical and laboratory), brain white matter hyper-intensities (Hazard Ratio [HR] 3.19, 95% Confidence Interval [CI] 1.02-9.91, p=0.04), anti-RNP positivity (HR 6.52, 95% CI 1.57-27.01, p=0.01), and low C4 levels (HR 3.80, 95% CI 1.22-11.83, p=0.02) remained significant (Table 3).

Conclusion: In primary aPL-positive patients with/without APS classification, based on 3,684 patient years of prospective follow-up, the incident SARD development rate was 5% (0.76 per 100 patient-years). Baseline white matter hyper-intensities, anti-RNP positivity, and low C4 levels, were associated with a new SARD development. Future registry analyses using standardized core laboratory lupus-related autoantibody results, and with longer follow-up times and larger numbers of new SARD, will help better define predictors of new SARD development in primary aPL/APS patients.

Supporting image 1

Table 1: Baseline Characteristics of Primary aPL/APS Patients Included in the APS ACTION Registry, Total and by New Systemic Autoimmune Rheumatic Disease (SARD) Development

Supporting image 2

Table 2: Associations Between Selected Baseline Characteristics and Development of a New SARD in Primary aPL/APS Patients Based on Univariate Analysis*

Supporting image 3

Table 3: Potential Predictors of Development of a New SARD in Primary aPL/APS Patients Adjusted for Each Other Based on Multivariate Analysis*

Disclosures: R. Kose Cobanoglu: None; R. Willis: Louisville APL Diagnostics Inc, 2, 8; D. Paredes-Ruiz: None; M. Tektonidou: None; V. Pengo: None; S. Sciascia: Chugai Pharmaceutical Co., Ltd., 2; C. Nalli: None; F. Signorelli: None; P. Fortin: AstraZeneca, 2, 6, GlaxoSmithKlein(GSK), 2, 6, Moderna, 2; M. Efthymiou: None; H. Belmont: Alexion, 1, Aurinia, 6; M. Petri: Amgen, 2, AnaptysBio, 2, Annexon Bio, 2, Arthros-FocusMedEd, 6, AstraZeneca, 2, 5, Atara Biosciences, 2, Aurinia, 5, 6, Autolus, 2, Avoro Ventures, 2, Biocryst, 2, Boxer Capital, 2, Cabaletto Bio, 2, Caribou Biosciences, 2, CTI, 1, CVS Health, 1, Eli Lilly, 2, 5, Emergent Biosolutions, 1, Ermiium, 2, Escient Pharmaceuticals, 2, Exagen, 5, Exo Therapeutics, 2, Gentibio, 2, GlaxoSmithKlein(GSK), 2, 5, iCell Gene Therapeutics, 2, Innovaderm Research, 2, IQVIA, 1, Janssen, 5, Kira Pharmaceuticals, 2, Merck/EMD Serono, 1, Nexstone Immunology, 2, Nimbus Lakshmi, 2, Novartis, 2, PPD Development, 2, Precision Biosciences, 2, Proviant, 2, Regeneron Pharmaceuticals, 2, Sanofi, 2, Seismic Therapeutic, 2, Senti Bioscienes, 2, Sinomab Biosciences, 2, Takeda, 2, Tenet Medicines Inc, 2, TG Therapeutics, 2, UCB, 2, Vertex Pharmaceuticals, 2, Worldwide Clinical Trials, 1, Zydus, 2; R. Cervera: None; M. Barber: AbbVie/Abbott, 2, AstraZeneca, 2, GlaxoSmithKlein(GSK), 2, Janssen, 2, Sanofi-Genzyme, 2; T. ATSUMI: None; C. Lopez-Pedrera: Eli Lilly, 5; J. Knight: ArgenX, 1, Visterra/Otsuka, 1, 2; D. Branch: UCB Pharma Inc, 5; N. Kello: None; L. Ji: None; E. Rodriguez-Almaraz: None; B. Artim Esen: None; J. Pardos Gea: None; R. Quintana: None; G. Pazzola: None; H. Shi: None; A. Duarte-Garcia: None; P. Meroni: None; R. Roubey: None; M. Bertolaccini: None; H. Cohen: argenx, 1, GlaxoSmithKlein(GSK), 6, Roche, 1, Technoclone (paid to Univ Coll London Hosp Charity), 6, UCB (paid to Univ College London Hosp Charity), 2; D. Andrade: None; D. Erkan: ACR, 5, APS ACTION, 4, Argenx, 1, Cadrenal, 2, Chugai, 1, EULAR, 5, Exagen, 5, GlaxoSmithKlein(GSK), 2, 5, 6, Kyverna, 1, NIH, 5, Otsuka/Visterra, 1, Up-To-Date, 9.

To cite this abstract in AMA style:

Kose Cobanoglu R, Willis R, Paredes-Ruiz D, Tektonidou M, Pengo V, Sciascia S, Nalli C, Signorelli F, Fortin P, Efthymiou M, Belmont H, Petri M, Cervera R, Barber M, ATSUMI T, Lopez-Pedrera C, Knight J, Branch D, Kello N, Ji L, Rodriguez-Almaraz E, Artim Esen B, Pardos Gea J, Quintana R, Pazzola G, Shi H, Duarte-Garcia A, Meroni P, Roubey R, Bertolaccini M, Cohen H, Andrade D, Erkan D. The Incident Risk and Predictors of Systemic Autoimmune Rheumatic Disease (SARD) Development in Persistently Antiphospholipid Antibody Positive Patients Without SARDs: Prospective Results from the APS ACTION Clinical Database and Repository (“Registry”) [abstract]. Arthritis Rheumatol. 2024; 76 (suppl 9). https://acrabstracts.org/abstract/the-incident-risk-and-predictors-of-systemic-autoimmune-rheumatic-disease-sard-development-in-persistently-antiphospholipid-antibody-positive-patients-without-sards-prospective-results-from-the-aps/. Accessed .

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