Abstracts tagged "Systemic sclerosis"

Abstract Number: 0046 • ACR Convergence 2025
Association of Genetic Variation in XIST and FTX with Susceptibility to Female-Biased Systemic Autoimmune Disease
Thomas Riley¹, Dana DiRenzo¹, Ellen Romich², Michael Levin³, Scott Damrauer³, Michael George¹, Montserrat Anguera¹, Joshua Baker¹ and Nikhil Jiwrajka¹, ¹University of Pennsylvania, Philadelphia, PA, ²University of Pennsylvania, Media, PA, ³University of Pennsylvania / Corporal Michael J. Crescenz VAMC, Philadelphia, PA
Background/Purpose: The mechanisms underlying female sex bias in autoimmune diseases remain unclear. Recent work has suggested that impaired maintenance of X-chromosome inactivation (XCI) in female…
Abstract Number: 2501 • ACR Convergence 2025
Microvascular dysfunction contributes to hand bone deterioration in SSc patients
Rosanna Campitiello¹, Sabrina Paolino², Andrea Casabella³, Giulia Davoli², Elisa Caratto⁴, Emanuele Gotelli¹, Elvis Hysa⁵, Carmen Pizzorni², Vanessa Smith⁶, Alberto Sulli² and Maurizio Cutolo⁷, ¹University of Genoa, Genoa, Liguria, Italy, ²University of Genoa, Genova, Italy, ³IRCCS Ospedale Policlinico San Martino, Genova, Italy, ⁴Univeristy of Genoa, Alessandria, Italy, ⁵University of Genoa, Genoa, Italy, ⁶Ghent University Hospital, Gent, Belgium, ⁷University of Genova, Genova, Italy
Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by endothelial dysfunction, self-reactive immune response and progressive systemic fibrosis1. It is well established that…
Abstract Number: 2475 • ACR Convergence 2025
GERD Severity, Proton Pump Inhibitor Use, and Longitudinal Forced Vital Capacity in the CONQUER Registry
Carrie Richardson¹, Shervin Assassi², Flavia Castelino³, Lorinda Chung⁴, Luke Evnin⁵, Tracy Frech⁶, Jessica Gordon⁷, Faye Hant⁸, Laura Hummers⁹, Dinesh Khanna¹⁰, Kimberly Lakin⁷, Dorota Lebiedz-Odrobina¹¹, Yiming Luo¹², Ashima Makol¹³, Maureen Mayes¹⁴, Zsuzsanna McMahan¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Nora Sandorfi¹⁸, Ami Shah⁹, Ankoor Shah¹⁹, Brian Skaug²⁰, Virginia Steen²¹, Elizabeth Volkmann²², Carleigh Zahn²³, John VanBuren¹¹ and Elana Bernstein²⁴, ¹Northwestern University, Chicago, IL, ²Division of Rheumatology, UTHealth Houston, Houston, Texas, USA, Houston, TX, ³Massachusetts General Hospital, Boston, MA, ⁴Stanford University, Stanford, CA, ⁵Scleroderma Research Foundation, Brisbane, CA, ⁶Vanderbilt University Medical Center, Nashville, TN, ⁷Hospital for Special Surgery, New York, NY, ⁸Medical University of South Carolina, Charleston, SC, ⁹Johns Hopkins Rheumatology, Baltimore, MD, ¹⁰University of Michigan, Ann Arbor, MI, ¹¹University of Utah, Salt Lake City, UT, ¹²Columbia University Irving Medical Center, New York, ¹³Mayo Clinic, Rochester, MN, ¹⁴UT Health Houston Division of Rheumatology, Houston, TX, ¹⁵UT Health Houston, Houston, TX, ¹⁶University of Minnesota, Minneapolis, MN, ¹⁷Northwestern University, Chicago, ¹⁸University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, ¹⁹Duke University, Durham, NC, ²⁰UTHealth Houston Division of Rheumatology, Houston, TX, ²¹Georgetown University School of Medicine, Washington, DC, ²²Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA, Los Angeles, CA, ²³University of Michigan, Ann Arbor, ²⁴Columbia University Irving Medical Center, New York, NY
Background/Purpose: Gastroesophageal reflux disease may contribute to the progression of interstitial lung disease in systemic sclerosis (SSc). However, it is unclear whether reflux severity or…
Abstract Number: 1865 • ACR Convergence 2025
Shared and unique molecular signatures across different autoantibody groups in systemic sclerosis: a multi-omics analysis
Hanlin Yin¹, Wanyi Lin², Zhangyi zhao¹, Chenhan Jia¹ and Liangjing Lu¹, ¹Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, Shanghai, China (People's Republic), ²Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China (People's Republic)
Background/Purpose: Antinuclear antibodies (ANA) are detected in over 95% of systemic sclerosis (SSc) patients. Compared to cutaneous subtype classification, autoantibody-based stratification more accurately predicts survival,…
Abstract Number: 1575 • ACR Convergence 2025
Clinical presentation, course, treatment and outcome of juvenile onset versus adult onset mixed connective tissue disease patients: a multicenter retrospective cohort.
Kevin Chevalier¹, Brigitte Bader-Meunier², Isabelle Kone-Paut³, Benjamin Torreau⁴, Marc Michel⁵, Bertrand Godeau⁵, Christian AGARD⁶, Thomas Papo⁷, Karim Sacré⁸, Raphaele Seror⁹, Xavier Mariette¹⁰, Cacoub Patrice¹¹, Ygal Benhamou¹², Mathilde Leclercq¹³, Cécile goujard¹⁴, Olivier Lambotte³, Bernard Bonnotte¹⁵, Maxime Samson¹⁶, Félix Ackermann¹⁷, Jean Schmidt¹⁸, Pierre Duhaut¹⁸, Jean-Emmanuel Kahn¹⁹, Thomas Hanslik¹⁹, Nathalie Costedoat-Chalumeau²⁰, Benjamin Terrier²⁰, Alexis REGENT²¹, bertrand Dunogue²², Pascal Cohen²³, Véronique Le Guern²⁰, Eric HACHULLA²⁴, Luc Mouthon²² and Benjamin Chaigne²², ¹Université Paris Cité, Montrouge, France, ²Necker hospital, Paris, France, ³Bicêtre hospital, Kremlin Bicêtre, France, ⁴Internal Medicine and Immunology, CHU Tours, Tours, France, ⁵Henri Mondor hospital, Créteil, France, ⁶Internal medicine, Nantes University Hospital, Nantes, France, ⁷Bichat hospital, Paris, France, ⁸Department of Internal Medicine, Bichat University Hospital, Université Paris Cité, AP-HP, Paris, France, Paris, France, ⁹Department of Rheumatology, National referral center for auto immune disease and Sjogren disease, Université Paris-Saclay, INSERM UMR¹¹⁸⁴: Centre for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique-Hôpitaux de Paris, Hôpital Bicêtre, Le Kremlin Bicêtre, Paris, France., le kremlin bicetre, France, ¹⁰Université Paris-Saclay, Le Kremlin Bicetre, France, ¹¹Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Centre national de références Maladies Autoimmunes et systémiques rares, Centre national de références Maladies Autoinflammatoires rares et Amylose inflammatoire (CEREMAIA), INSERM, UMR S⁹⁵⁹, Immunology-Immunopathology-Immunotherapy (I³), Paris, France, Paris, France, ¹²Internal Medicine, CHU Rouen, Rouen, France, ¹³Rouen hospital, Rouen, France, ¹⁴Université Paris Saclay, Department of Internal Medicine and Clinical Immunology, Bicêtre Hospital, APHP, UMR¹¹⁸⁴ Inserm, CEA, Le Kremlin Bicêtre, France, Kremlin Bicêtre, France, ¹⁵Internal medicine and clinical immunology, Université Bourgogne Europe , CHU Dijon Bourgogne, Dijon, France, ¹⁶CHU Dijon Bourgogne, Dijon, France, ¹⁷Foch hospital, Suresnes, France, ¹⁸Amiens hospital, Amiens, France, ¹⁹Ambroise Paré hospital, Boulogne, France, ²⁰Cochin hospital, Paris, France, ²¹Hopital Cochin, Paris, France, ²²Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin University Hospital, Université Paris Cité, AP-HP, Paris, France, ²³Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Hospital Cochin, Paris, France, ²⁴CHU Lille, Département de Médecine Interne et Immunologie Clinique, Centre de Référence des Maladies Auto-Immunes et Auto-Inflammatoires Rares du Nord, Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), Lille, France, Lille, France
Background/Purpose: Mixed connective tissue disease (MCTD) is an entity defined by clinical features of differentiated connective tissue diseases (dCTD), such as systemic lupus erythematosus (SLE),…
Abstract Number: 1193 • ACR Convergence 2025
Lung Transplant Outcomes in Patients with Myositis- and Systemic Sclerosis-Associated Interstitial Lung Disease Compared to Idiopathic Pulmonary Fibrosis: A Multicentric Retrospective Analysis
Angela Chang¹, Navid Saleh¹, Alec Yu¹, Darya S. Jalaledin², Sabrina Hoa³, Robert Levy⁴, Jennifer Wilson⁵, Charles Poirier⁶, John Yee¹, James Choi¹, Océane Landon-Cardinal⁷, Hyein Kim⁸ and Kun Huang⁹, ¹University of British Columbia, Vancouver, BC, Canada, ²Université de Montréal, Saint-Lambert, QC, Canada, ³University of Montreal, Brossard, QC, Canada, ⁴University of British Columbia, Deerfield, IL, ⁵UBC, Vancouver, BC, Canada, ⁶University of Montreal, Montreal, QC, Canada, ⁷Centre hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁸The University of British Columbia, Vancouver, BC, Canada, ⁹University of British Columbia, Vancouver, Surrey, BC, Canada
Background/Purpose: Interstitial lung disease (ILD) is a frequent complication of idiopathic inflammatory myositis (IIM) and systemic sclerosis (SSc), ranging from subclinical ILD to rapidly progressive…
Abstract Number: 0961 • ACR Convergence 2025
Retinoic acid-related orphan receptor-α: A novel upstream regulator of Hippo signaling and potential therapeutic target in fibrosis
Cuong Tran-Manh¹, Thuong Trinh-Minh², Christoph Liebel³ and Jörg Distler⁴, ¹Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University., Düsseldorf, Nordrhein-Westfalen, Germany, ²Clinic for Rheumatology University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany; Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Nordrhein-Westfalen, Germany, ³Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University., Düsseldorf, Germany, ⁴University Hospital Duesseldorf and HHU, Duesseldorf, Germany
Background/Purpose: Retinoic acid-related orphan receptor-α (RORα) is a nuclear transcription factor implicated in immune regulation, circadian rhythm, and metabolism. However, its role in fibrotic diseases…
Abstract Number: 0688 • ACR Convergence 2025
Total lung capacity is predictive of disease severity and survival in systemic sclerosis: a longitudinal analysis in 2347 patients from the French National Cohort Study
Benjamin Chaigne¹, Alexandre bense², Frédérique Aubourg³, Christian AGARD⁴, Yannick Allanore⁵, Alice berezne¹, Gregory Pugnet⁶, Eric Hachulla⁷, Vincent Cottin⁸, Arnaud Hot⁸, bertrand Dunogue¹, Anuxcy Kanagaratnam², Sylvain Palat⁹, Alain Lescoat¹⁰, Sabine berthier¹¹, Emmanuel Chatelus¹², Sébastien Rivière¹³, David Launay¹⁴, Marie-Elise Truchetet¹⁵, Anh-Tuan Dinh-Xuan³ and Luc Mouthon¹, ¹Department of Internal Medicine, National Referral Center for Rare Systemic Autoimmune Diseases, Cochin University Hospital, Université Paris Cité, AP-HP, Paris, France, ²AP HP, Paris, France, ³Unité exploration fonctionnelle respiratoire, Pneumology department, Cochin Hospital, APHP, PARIS, France, ⁴Internal medicine, Nantes University Hospital, Nantes, France, ⁵Université Paris Cité, Paris, France, ⁶CHU, Montpelliers, France, ⁷CHU, Lilles, France, ⁸CHU, Lyon, France, ⁹Limoges, Limoges, France, ¹⁰CHU Rennes - University Rennes, Rennes, France, ¹¹CHU, Dijon, France, ¹²Department of Rheumatology, University Hospital Strasbourg, France, Strasbourg, France, ¹³AP HP, Parsi, France, ¹⁴Univ. Lille, Inserm, CHU Lille, Department of Internal Medicine and Clinical Immunology, Reference Center for Rare Autoimmune and Autoinflammatory diseases (CERAINOM), U¹²⁸⁶ - INFINITE - Institute for Translational Research in Inflammation, Lille, France. National Reference Center for Pulmonary Arterial Hypertension (Pulmotension), Lille, France, Lille Cedex, France, ¹⁵Bordeaux University Hospital, Bordeaux, France
Background/Purpose: Total lung capacity (TLC) is seldom assessed in the prediction of systemic sclerosis (SSc) disease severity.Herein, we utilized the French SSc national database to…
Abstract Number: 0884 • ACR Convergence 2025
Risk Score for Early Mortality to stratify for Intensive SSc Therapy
Ann-Christin Pecher¹, Boubaya Marouane², Oliver Distler³, Vanessa Smith⁴, Jeska de Vries-Bouwstra⁵, Radim Bečvář⁶, Gianluca Moroncini⁷, David Launay⁸, Yannick Allanore⁹, Maria De Santis¹⁰, Kamal Solanki¹¹, Carlomaurizio Montecucco¹², Luca Idolazzi¹³, Nihal Fathi¹⁴, Przemyslaw Kotyla¹⁵, Muriel Elhai¹⁶ and Jörg Henes¹⁷, ¹Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Unit of Clinical Research, Paris Seine Saint Denis University, Bobigny, France, Paris, France, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland, ⁴Ghent University Hospital, Gent, Belgium, ⁵Leiden University Medical Center, Leiden, Netherlands, ⁶Department of Internal Medicine, Charles University,, Prague, Czech Republic, ⁷Department of Internal Medicine, Marche University Hospital, Clinica Medica, Ancona, Italy, Ancona, Italy, ⁸Univ. Lille, Inserm, CHU Lille, U¹²⁸⁶ – INFINITE – Institute for Translational Research in Inflammation, Lille, France ; CHU Lille, Département de Médecine interne et Immunologie Clinique, Centre de Référence des Maladies Auto-immunes Systémiques Rares du Nord et Nord-Ouest, Méditerranée et Guadeloupe (CeRAINOM), Lille France, Lille, France, ⁹Department of Rheumatology, Université Paris Cité UFR de Médecine, Paris, France, ¹⁰Humanitas University, Pieve Emanuele, Italy, ¹¹Waikato Hospital, Hamilton, New Zealand, ¹²Department of Internal Medicine and Therapeutics, Università di Pavia, Pavia, Italy, Pavia, Italy, ¹³Rheumatology Section, Department of Medicine, University of Verona, Verona, Italy, Verona, Italy, ¹⁴Department of Rheumatology ,Rehabilitation & physical medicine, Assiut University Hospital, Assiut, Egypt, Assiut, Egypt, ¹⁵Department of Internal Medicine, Rheumatology and Clinical Immunology, Medical University of Silesia, Katowice, Poland, ¹⁶University Hospital zurich, Zürich, Switzerland, ¹⁷Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tuebingen, Germany
Background/Purpose: Systemic sclerosis (SSc) is a rare disease that often leads to severe complications and premature mortality. Recent advancements in the field have led to…
Abstract Number: 0032 • ACR Convergence 2025
Protein Language Model-Guided Homology Identifies Microbial Enzymes Linked to Fibrosis-Prone IgG4-RD and Crohn’s Disease
Kumar Thurimella¹, Ahmed Mohamed², Chenhao Li³, Tommi Vatanen⁴, Daniel Graham³, Roisin Owens⁵, Sabina Leanti La Rosa⁶, Damian Plichta³, Sergio Bacallado⁵ and Ramnik Xavier⁷, ¹University of Colorado School of Medicine, Aurora, CO, ²Broad Institute, Boston, ³Broad Institute, Cambridge, MA, ⁴University of Helsinki, Helsinki, Finland, ⁵University of Cambridge, Cambridge, United Kingdom, ⁶NMBU, As, Norway, ⁷Harvard Medical School, Boston, MA
Background/Purpose: Uncharacterized microbial enzymes in metagenomics are difficult to annotate, especially in fibrosis-prone conditions like IgG4-related disease (IgG4-RD) and Crohn’s disease (CD), where microbial carbohydrate…
Abstract Number: 2500 • ACR Convergence 2025
Greater Economic Burden and Healthcare Resource Utilization in Patients with Systemic Sclerosis With Versus Without Interstitial Lung Disease: Results from a Systematic Literature Review
Emily Glowienka¹, Sarah Nickolls², Kevin Coady¹, Eesha Kodi³, Ian Steinfield¹, Florence Wilson³, Alicia N. Pepper³, Svetlana I. Nihtyanova⁴, Roger A. Levy⁵ and Daniel Moldaver⁶, ¹Precision AQ, Boston, MA, ²GSK, Research & Development, Stevenage, United Kingdom, ³Precision AQ, Vancouver, BC, Canada, ⁴GSK, Clinical Sciences, Research & Development, London, United Kingdom, ⁵GSK, Specialty Care, Global Medical Affairs, Collegeville, PA, ⁶GSK, Global Real-World Evidence & Health Outcomes Research, Toronto, ON, Canada
Background/Purpose: SSc is a rare autoimmune connective tissue disorder; up to 64% of patients with SSc develop interstitial lung disease (ILD), which can result in…
Abstract Number: 2474 • ACR Convergence 2025
Unveiling the clinical spectrum of ACA-positive SSc-ILD: not as benign as expected
Carlos Valera Ribera¹, JUAN JOSE ALEGRE SANCHO², Ivan Castellvi³, Marta Ibañez⁴ and Javier Narváez⁵, ¹Hospital Universitario Doctor Peset, València, Spain, ²Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ³Hospital Universitari de la Santa Creu i Sant Pau, Sant Just Desvern, Spain, ⁴Hospital Universitario de la Santa Creu i Sant Pau, Barcelona, Catalonia, Spain, ⁵Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Anticentromere antibody (ACA)-positive interstitial lung disease (ILD) in systemic sclerosis (SSc) is traditionally considered less aggressive than anti-topoisomerase I (ATA)-positive ILD. However, its clinical…
Abstract Number: 1864 • ACR Convergence 2025
Mitochondrial Dysfunction Drives cGAS-STING–Mediated Type I Interferon Production and Fibrosis in Systemic Sclerosis
Giulio Forte¹, Vasiliki Liakouli¹, Alessia Salzillo², Mario Angeli³, Daniele Mauro¹, Antonio Ciancio¹, Barbara De Marino², Iacopo Panarese¹, Mario Faenza¹, roberto giacomelli⁴, Andreas Ramming⁵ and Francesco Ciccia⁶, ¹Università della Campania Luigi Vanvitelli, Naples, Italy, ²University of Campania L. Vanvitelli, Naples, Italy, ³Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ⁴Università Campus Bio-Medico, Roma, Italy, ⁵Department of Internal Medicine ³, Rheumatology & Immunology, Friedrich-Alexander-University (FAU) & Universitätsklinikum Erlangen, Erlangen, Germany, ⁶Rheumatology Section, University of Campania L. Vanvitelli, Naples, Italy, Naples, Italy
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by type I interferon (IFN-I) production and mitochondrial dysfunction. Emerging evidence suggests that activation of the…
Abstract Number: 1573 • ACR Convergence 2025
Health-related quality of life over 15 years in systemic sclerosis: impact of sex and survival
Katherine van der Wouden¹, Georgy Gomon², Rachel Knevel², Michel Tsang-A-Sjoe³, Alexandre Voskuijl³ and Jeska de Vries-Bouwstra², ¹Leiden University Medical Center and Amsterdam University Medical Center, Leiden, Zuid-Holland, Netherlands, ²Leiden University Medical Center, Leiden, Netherlands, ³Amsterdam University Medical Center, Amsterdam, Netherlands
Background/Purpose: Patients with systemic sclerosis (SSc) face an uncertain long‑term outlook; understanding how their health‑related quality of life (HRQoL) changes over time can help them…
Abstract Number: 1161 • ACR Convergence 2025
Comparative efficacy, safety and tolerability of antifibrotic therapies across systemic autoimmune diseases
Javier Narváez¹, Orfa Barrios², Pol Maymó-Paituvi³, JUAN JOSE ALEGRE SANCHO⁴, Ivan Castellví⁵, Vanesa Vicens Zygmunt⁶, Guadalupe Bermudo⁷, Laia De Daniel Bisbe³, Martí Aguilar-Coll⁸, Montserrat Roig Kim³, Joan Miquel Nolla³ and Maria Molina-Molina⁷, ¹Hospital Universitario de Bellvitge, Barcelona, Spain, ²Department of Rheumatology. Hospital Nacional Arzobispo Loayza, Lima, Peru, ³Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, ⁴Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, ⁵Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, barcelona, Spain, ⁶Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁷Interstitial Lung Disease Unit. Department of Pneumology. Hospital Universitario de Bellvitge., Barcelona, Spain, ⁸Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: To evaluate the efficacy, safety, and tolerability of the antifibrotic agents nintedanib and pirfenidone in the treatment of interstitial lung disease (ILD) associated with…

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