Abstracts tagged "Systemic sclerosis"

Abstract Number: 1725 • ACR Convergence 2025
Functional NOTCH4 Variants Drive Vasculopathy and Fibrosis in Systemic Sclerosis.
Urvashi Kaundal¹, Pei-Suen Tsou², Mousumi Sahu³, Mengqi Huang⁴, Steven Boyden⁵, Curtis Woodford⁶, Daniel Shriner⁷, Emilee Stenson⁴¹, Sarah Safran⁸, Yuechen Zhou⁹, Taylor Talley⁴², Kaavya Gudapati⁴³, Xuetao Zhang⁶, Yosuke Kunishita¹⁰, Janet Wang⁴⁴, Ami Shah¹¹, Maureen Mayes¹², Ayo Doumatey¹³, Amy Bentley⁷Janet , Robyn Domsic⁴, Thomas Medsger, Jr¹⁴, Paula Ramos¹⁵, Richard Silver¹⁶, Virginia Steen¹⁷, John Varga², Vivien Hsu¹⁸, Lesley Ann Saketkoo¹⁹, Elena Schiopu²⁰, Jessica Gordon²¹, Lindsey Criswell²², Heather Gladue²³, Chris Derk²⁴, Elana Bernstein²⁵, S. Louis Bridges²¹, Victoria Shanmugam²⁶, Lorinda Chung²⁷, Suzanne Kafaja²⁸, Reem Jan⁴⁵, Marcin TROJANOWSKI²⁹, Avram Goldberg⁴⁶, Benjamin Korman³⁰, James Mullikin³¹, James Thomas³¹, Stefania Dell'orso³², davide Randazzo³³, Adebowale Adeyemo⁷, Elaine Remmers³⁴, Pamela Schwartzberg³⁵, Ivona Aksentijevich³⁶, Charles Rotimi⁷, Fredrick Wigley³⁷, Rong Wang⁶, Francesco Boin³⁸, Dinesh Khanna², Robert Lafyatis⁴, Daniel Kastner³⁹, Pravitt Gourh⁴⁰, ¹National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Chevy Chase, MD, ²University of Michigan, Ann Arbor, MI, ³National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ⁴University of Pittsburgh, Pittsburgh, PA, ⁵Utah Center for Genetic Discovery, Department of Human Genetics, University of Utah, Salt Lake City, UT, Bethesda, MD, ⁶Laboratory for Accelerated Vascular Research, Department of Surgery, University of California, San Francisco, San Francisco, CA, san francisco, CA, ⁷Center for Research on Genomics and Global Health, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, bethesda, MD, ⁸National Institute of Arthritis and Musculoskeletal and Skin Diseases, New York, NY, ⁹Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, Pittsburg, PA, ¹⁰National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Rockville, MD, ¹¹Johns Hopkins Rheumatology, Baltimore, MD, ¹²UT Health Houston Division of Rheumatology, Houston, TX, ¹³Center for Research on Genomics and Global Health, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, bethedsa, MD ¹⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, Verona, PA, ¹⁵Emory University School of Medicine, Atlanta, GA, ¹⁶Medical University of South Carolina, Charleston, SC ¹⁷Georgetown University School of Medicine, Washington, DC, ¹⁸Rutgers- RWJ Medical School, South Plainfield, NJ, ¹⁹University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, ²⁰Division of Rheumatology, Medical College of Georgia at Augusta University, Augusta, GA, Augusta, GA, ²¹Hospital for Special Surgery, New York, NY, ²²NIH/NHGRI, Bethesda, MD, ²³Arthritis & Osteoporosis Consultants of the Carolinas, Charlotte, NC, ²⁴University of Pennsylvania, Philadelphia, PA, ²⁵Columbia University Irving Medical Center, New York, NY, ²⁶Office of Autoimmune Disease Research, Office of Research on Women's Health, Office of the Director, National Institutes of Health, Bethesda, MD, bethesda, MD, ²⁷Stanford University, Stanford, CA, ²⁸UCLA Department of Medicine, Division of Rheumatology, Los Angeles, CA, ²⁹BOSTON UNIVERSITY SCHOOL OF MEDICINE, BOSTON, MA, ³⁰University of Rochester, Rochester, NY, ³¹NIH Intramural Sequencing Center, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, bethesda, MD, ³²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³³Light Imaging Section, Office of Science and Technology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, ³⁴Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD, bethesda, MD, ³⁵Cell Signaling and Immunity Section, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ³⁶100, Bethesda, MD, ³⁷Johns Hopkins University, Baltimore, MD, ³⁸Cedars-Sinai Medical Center, Beverly Hills, CA, ³⁹National Human Genome Research Institute, Bethesda, MD, ⁴⁰National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD,⁴¹NIH, Bethesda, MD, ⁴² Spelman College and post-bac National Institutes of Health, Bethesda, MD, ⁴³Georgia Institute of Technology, Alpharetta, GA, ⁴⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Beachwood, OH, ⁴⁵University of Chicago, Chicago, IL, ⁴⁶NYU Langone Health - NYU Hospital for Joint Diseases, Lake Success, NY.

Background/Purpose: Systemic sclerosis (SSc) is characterized by vasculopathy, progressive fibrosis of skin and internal organs, and autoimmunity. Notably, African American (AA) patients with SSc exhibit…
Abstract Number: 1567 • ACR Convergence 2025
Deep-learning analysis of HRCT images predicts progression and mortality in systemic sclerosis-related interstitial lung disease
Enrico De Lorenzis¹, Rosa D'Abronzo², Pier Giacomo Cerasuolo³, Lucio Calandriello⁴, Gabriella Alonzi³, Giuseppe Cicchetti², gerlando Natalello³, Bruno Iovene⁵, Lucia Lanzo³, Francesco Varone⁶, Giacomo Sgalla⁷, Luca Richeldi⁸, anna Rita Larici⁴, Maria Antonietta D'Agostino³ and Silvia Laura Bosello⁹, ¹Catholic University of the Sacred Heart, Roma, Rome, Italy, ²Division of Radiology - Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy, ³Division of Rheumatology and Clinical Immunology - Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ⁴Division of Radiology - Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ⁵Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ⁶Division of Respiratory Medicine - Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ⁷Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ⁸Division of Respiratory Medicine - Università Cattolica del Sacro Cuore, Fondazione Policlinico Universitario A. Gemelli IRCCS, Roma, Italy, ⁹Division of Rheumatology and Clinical Immunology - Catholic University of the Sacred Heart, Rome, Italy, Rome, Italy
Background/Purpose: Interstitial lung disease (ILD) is a major complication in systemic sclerosis (SSc) patients, associated with substantial morbidity and mortality. Functional, imaging, and clinical measures…
Abstract Number: 0992 • ACR Convergence 2025
FoxP3Lo CD4+ T cells are functionally suppressive and expanded in the immune-mediated fibrotic diseases IgG4-related disease and systemic sclerosis
Laura J. Yockey¹, Ian Doyle², Thomas Guy³, Devanshi Trivedi², Chinmay Gadiraju⁴, Federica Bonaso⁵, Jesse Akaa², Alison Puri⁶, Zachary Wallace⁷, Guy Katz⁸, Sydney Montesi⁸, John Stone⁹, Flavia Castelino⁸, Shiv Pillai¹⁰, Andrew Luster¹¹, Vinay S. Mahajan¹² and Cory Perugino¹³, ¹MGH, Charlestown, MA, ²Massachusetts General Hospital (MGH - Mass General) (MGB), Boston, MA, ³Ragon Institute of MGH, MIT and Harvard/ Royal Prince Alfred Hospital Sydney, Sydney, Australia, ⁴Ragon Institute of MGH, MIT and Harvard, Cambridge, MA, ⁵University of Brescia, ASST Spedali Civili of Brescia, Massachusetts General Hospital, Monza, Italy, ⁶Boston University, Brookline, MA, ⁷Massachusetts General Hospital, Newton, MA, ⁸Massachusetts General Hospital, Boston, MA, ⁹Massachusetts General Hospital , Harvard Medical School, Concord, MA, ¹⁰Harvard Medical School, Cambridge, MA, ¹¹Massachusetts General Hospital, Charlestown, MA, ¹²Brigham and Women's Hospital, Boston, MA, ¹³Massachusetts General Hospital, Harvard Medical School, Boston, MA
Background/Purpose: The absence of regulatory T cells (Tregs) results in multiorgan autoimmunity in the context of monogenic “Tregopathies,” but their role in mediating polygenic autoimmune…
Abstract Number: 0955 • ACR Convergence 2025
Surveying RNA methylation in scleroderma highlights roles for demethylases ALKBH5 and FTO in fibrosis
Alexander Cai¹, Alyssa Rosek¹, Neha Khanna¹, Anna Webber¹, Karly Kozicki¹, Dinesh Khanna² and Pei-Suen Tsou², ¹Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, ²University of Michigan, Ann Arbor, MI
Background/Purpose: Recent studies indicate that genes involved in RNA methylation may play a significant role in cellular functions, and disruptions in RNA methylation have been…
Abstract Number: 0682 • ACR Convergence 2025
Predictors of Severe Outcomes in Scleroderma Renal Crisis: Updated Results from a Single Center Study
Alex Luta¹, Saloni Mitchell² and Virginia Steen³, ¹Hospital for Special Surgery, New York, NY, ²Mount Sinai West and Morningside, New York, NY, ³Georgetown University School of Medicine, Washington, DC
Background/Purpose: Scleroderma Renal Crisis (SRC) is a severe manifestation of Systemic Sclerosis (SSc) associated with elevated morbidity and mortality. We aimed to address the challenges…
Abstract Number: 0847 • ACR Convergence 2025
Interferon Score Effectively Stratifies for Time to Clinically Significant Event Accrual in Systemic Sclerosis Independently of Cutaneous Subset
Stefano Di Donato¹, Rebecca Ross², Marco Minerba³, Vishal Kakkar⁴, Enrico De Lorenzis⁵, Philip Yee⁶, Christopher Denton⁷ and Francesco Del Galdo⁴, ¹University of Leeds, Canosa Sannita, Chieti, Italy, ²Medicine and Health, University of Leeds, Leeds, United Kingdom, ³Campus Bio-Medico University of Rome, Taranto, Taranto, Italy, ⁴University of Leeds, Leeds, United Kingdom, ⁵Catholic University of the Sacred Heart, Roma, Rome, Italy, ⁶UCL, London, United Kingdom, ⁷University College London, UK, London, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is a heterogeneous autoimmune disease characterized by vascular damage, immune dysregulation, and fibrosis. It is typically classified into limited cutaneous (lcSSc)…
Abstract Number: 2491 • ACR Convergence 2025
High Prevalence of Autoimmunity and Cancer in Anti-NOR90-positive Patients: A Multicenter Observational Study
Marina Dueñas-Ochoa¹, Cristina Valero², Francisco Morandeira³, Juan Carlos Sáez¹, Maryia Nikitsina⁴, Montserrat Roig Kim⁵, Laia De Daniel Bisbe⁵, Esther Vicente-Rabaneda⁶, Arantza Alfranca¹, Miguel A. González-Gay⁷, Martí Aguilar Coll⁸, Rosario Garcia Vicuña⁶, Javier Narváez⁹ and Santos Castañeda¹⁰, ¹Hospital La Princesa, Madrid, Spain, ²Hospital de la Princesa, Madrid, Spain, ³Department of Immunology. Hospital Universitario de Bellvitge, Barcelona, Spain, ⁴University Hospital La Princesa, Madrid, Madrid, Madrid, Spain, ⁵Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, ⁶Hospital Universitario de La Princesa, Madrid, Spain, ⁷Department of Rheumatology and Joint and Bone Research Unit, Hospital Universitario Fundación Jiménez Díaz, and Instituto de Investigación Sanitaria-Fundación Jiménez Díaz, Madrid, Spain, and Medicine and Psychiatry Department, University of Cantabria, Santander, Spain, ⁸Hospital Bellvitge, Barcelona, Spain, ⁹Hospital Universitario de Bellvitge, Barcelona, Spain, ¹⁰Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Madrid, Spain
Background/Purpose: Anti-Nucleolar Organizer Region 90 (anti-NOR90) antibodies target nucleolar proteins involved in ribosomal RNA transcription and processing, key steps in protein synthesis. These antibodies have…
Abstract Number: 2017 • ACR Convergence 2025
Baseline and 2-year Follow-up of Pulmonary Function Among CTD-ILD Subtypes
Ivana Ilic, Anchal Sabharwal, Galina Marder and Sonali Narain, Northwell, Great Neck
Background/Purpose: Interstitial lung disease is a major cause of morbidity and mortality in patients with connective tissue disease (CTD-ILD). Evaluation of ILD severity and progression…
Abstract Number: 1588 • ACR Convergence 2025
Prevalence Of Oral Manifestations And Their Association With Clinical And Serological Profile In Systemic Sclerosis Patients- An Indian Study
Vijaya prasanna Parimi¹, Tejaswini Ramineni², Pradeep S Anand³, Vineeta Shobha⁴, Padmanabha Shenoy⁵, Geetabali Sircar⁶, kaushik Basu⁷, Anna C Das⁴, Geetha Amritrao Kale⁴, indranil sarkar⁸, Shinie Razil Goveas⁴, Biswarup Sengupta⁸, Caseena Kareem⁹, Yogananth Sakthivel⁴, Soma Halder Biswas⁷ and Neel Nallulwar⁸, ¹ESIC Medical College and Super Specilaity Hospital, hyderabad, Telangana, India, ²Esic Medical College And Hospital, Sanathnagar, Hyderabad, Telangana, India, ³ESIC MEDICAL COLLEGE AND HOSPITAL, HYDERABAD, Telangana, India, ⁴St. John's Medical College Hospital, Bangalore, Bangalore, Karnataka, India, ⁵Shenoy's CARE, Kochi, Kerala, India, ⁶West Bengal Medical Education Service, Kolkata, West Bengal, India, ⁷Medical College, Kolkata, kolkata, West Bengal, India, ⁸IPGMER, Kolkata, kolkata, West Bengal, India, ⁹CARE, Kochi, kochi, Kerala, India
Background/Purpose: Systemic sclerosis (SSC) is a chronic, multisystem autoimmune disease characterized by immune dysfunction, microangiopathy, and tissue remodelling. Orofacial manifestations, including xerostomia, microstomia, tooth decay,…
Abstract Number: 1564 • ACR Convergence 2025
Validation of a Composite Endpoint for Systemic Sclerosis-Associated Interstitial Lung Disease
Elizabeth Volkmann¹, Holly Wilhalme², Samuel Good², Grace Kim², Jonathan Goldin², Michael Roth² and Donald Tashkin², ¹Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA, Los Angeles, CA, ²UCLA, Los Angeles
Background/Purpose: The forced vital capacity (FVC) is the most commonly used endpoint in registrational trials for systemic sclerosis-associated interstitial lung disease (SSc-ILD). However, the FVC…
Abstract Number: 0975 • ACR Convergence 2025
Linezolid prevents fibroblast activation and ameliorates tissue fibrosis by inhibition of mitochondrial translation
Xuezhi Hong¹, Yanhua Xiao², shihao zhu³, Tim Filla⁴, Andrea-Hermina Györfi⁵, Yi-Nan Li⁶, Meilin Xu⁷, Langxian Zhi², Thuong Trinh-Minh⁸, Clara Dees⁹, Georg Schett¹⁰, Jörg Distler¹¹ and Alexandru-Emil Matei¹², ¹Department of Rheumatology, University Hospital Dusseldorf, Medical Faculty of Heinrich Heine University, Dusseldorf, Germany, Düsseldorf, Germany, ²Medical Faculty of Heinrich Heine University, Dusseldorf, Germany, ³Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, ⁴Department 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, ⁵Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University., Düsseldorf, Germany, ⁶University Hospital of Düsseldorf, Düsseldorf, Germany, ⁷Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University, Düsseldorf, Germany, Düsseldorf, Germany, ⁸Clinic 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, ⁹Department of Internal Medicine ³, Rheumatology and Clinical Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany, Erlangen, Germany, ¹⁰Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, ¹¹University Hospital Duesseldorf and HHU, Duesseldorf, Germany, ¹²Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer Cluster of Excellence for Immune Mediated Diseases CIMD, Frankfurt am Main, Germany, Düsseldorf, Germany
Background/Purpose: Systemic sclerosis (SSc) is a progressive fibrotic disease characterized by fibroblast activation and immune dysregulation, with limited therapeutic options. Mitochondrial dysfunction is increasingly implicated…
Abstract Number: 0709 • ACR Convergence 2025
Systemic Sclerosis Inducible Pluripotential Stem Cells Reprogrammed into Endothelial Cells Identify Vascular Permeability May Not Be Cytokine Driven
Tracy Frech¹, Venkateswara Gogulamudi², Denisse Moreno², Eric Austin¹, Charles Frech³, Anna Hemnes¹, Tania Ruiz¹, Alex Shahin¹, Megha Talati¹, Colin Maguire⁴ and Anthony Donato², ¹Vanderbilt University Medical Center, Nashville, TN, ²Salt Lake Veterans Affairs Medical Center, Salt Lake City, UT, ³Vanderbilt University, Nashville, TN, ⁴University of Utah, Salt Lake City, UT
Background/Purpose: Systemic sclerosis (SSc) disease models that faithfully recapitulate endothelial mediated vasculopathy are critical for testing and developing novel treatments. The generation of inducible pluripotential…
Abstract Number: 0680 • ACR Convergence 2025
Altered miRNA patterns of peripheral blood mononuclear cells show correlations with pulmonary manifestations of systemic sclerosis
Dóra Csige, Szilárd Póliska, János Rózsa, Dóra Tari, Szilvia Szamosi, Zoltán Szekanecz, Gabriella Szűcs and Levente Bodoki, University of Debrecen, Debrecen, Hungary
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune rheumatic and musculoskeletal disease (RMD) with a high mortality. Epigenetic factors, such as miRNAs might play an important…
Abstract Number: 0846 • ACR Convergence 2025
A Longitudinal Transcriptomic Study of Mycophenolate Mofetil in Systemic Sclerosis Skin with Clinical and Molecular Stratification
Rezvan Parvizi¹, Zhiyun Gong², Natania Field³, Helen Jarnagin², Dillon Popovich², Monica Yang⁴, Kathleen Aren⁵, Mary Carns⁶, Isaac Goldberg⁷, Lorinda Chung⁸, Vivien Goh⁹, Zsuzsanna McMahan¹⁰, Tammara Wood¹¹, Dinesh Khanna¹², Monique Hinchcliff¹³ and Michael Whitfield¹⁴, ¹Dartmouth, lebanon, NH, ²Dartmouth College, Lebanon, NH, ³Northwestern Memorial Hospital, Chicago, IL, ⁴UCSF, San Francisco, CA, ⁵Northwestern University Division of Rheumatology, Chicago, IL, ⁶Northwestern University, Chicago, IL, ⁷Wayne State University, Detroit, ⁸Stanford University, Stanford, CA, ⁹Northwestern, Chicago, ¹⁰UT Health Houston, Houston, TX, ¹¹Dartmouth, Hanover, NH, ¹²University of Michigan, Ann Arbor, MI, ¹³Yale School of Medicine, Westport, CT, ¹⁴Geisel School of Medicine, Lebanon, NH
Background/Purpose: Mycophenolate Mofetil (MMF) is the most commonly prescribed immunosuppressive treatment for patients diagnosed with diffuse cutaneous systemic sclerosis (dcSSc). Here, we analyzed skin gene…
Abstract Number: 2490 • ACR Convergence 2025
Unmasking Mortality Risk: The Impact of Systemic Sclerosis in Stress-Induced Cardiomyopathy
Suryakumar Balasubramanian¹, Laura Rojo Grajales², Ashlyn Narman³, Veera Durga Vaishnavi Kurra⁴, Ramses Ramirez Damera², Alejandro Alvarez Betancourt², Abdul Qudoos Iqbal Mohammed², Hai Ofek², Prachi Anand², Roman Zeltser⁵ and Amgad N. Makaryus⁵, ¹Nassau university Medical center, new york, ²Nassau University Medical Center, East Meadow, NY, ³American University of the Caribbean, East Meadow, NY, ⁴University of Oklahoma Health Sciences Center, Oklahoma City, OK, ⁵Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Hempstead, NY
Background/Purpose: Stress-induced cardiomyopathy (SIC), also known as Takotsubo cardiomyopathy (TCM), is characterized by acute left ventricular dysfunction, often triggered by emotional or physical stress. In…

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