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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 2485 • ACR Convergence 2025

    Prevalence of progressive pulmonary fibrosis in systemic sclerosis-associated interstitial lung disease

    Javier Narváez1, Irene Carrión-Barberà2, Carlos Valera Ribera3, Alfredo Guillen del Castillo4, Laura Tio5, Laura Triginer6, Lidia Montala-Valencia7, Carmen Pilar Simeon8, Anna Pros2, JUAN JOSE ALEGRE SANCHO9 and Joan Miquel Nolla10, 1Hospital Universitario de Bellvitge, Barcelona, Spain, 2Department of Rheumatology. Hospital del Mar, Barcelona, Spain, 3Hospital Universitario Doctor Peset, València, Spain, 4Departmend of Internal Medicine. Hospital Universitario Vall d´Hebron, Barcelona, Spain, 5Hospital del Mar Research Institute, Barcelona, Spain, 6Vall d´Hebron Institut de Recerca (VHIR), Barcelona, Spain, 7Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain, 8Department of Internal Medicine, Hospital Universitario Vall d’Hebron, Barcelona, Spain, 9Department of rheumatology. Hospital Universitario Doctor Peset, Valencia, Spain, 10Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain

    Background/Purpose: To estimate the prevalence of the progressive pulmonary fibrosis (PPF) phenotype among patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD) and identify risk factors…
  • Abstract Number: 1861 • ACR Convergence 2025

    Accelerated and Gene-Specific Patterns of Clonal Hematopoiesis Distinguish Subtypes of Systemic Sclerosis

    Brecca Miller1, Shervin Assassi2, Maureen Mayes3, alejandro Molina4, Kelly Ruggles5, David Beck6 and Meng Zhang7, 1Center for Human Genetics and Genomics, NYU Grossman School of Medicine, Brooklyn, NY, 2Division of Rheumatology, UTHealth Houston, Houston, Texas, USA, Houston, TX, 3UT Health Houston Division of Rheumatology, Houston, TX, 4Georgia Institute of Technology, Atlanta, GA, 5NYU Grossman School of Medicine, Brooklyn, NY, 6Center for Human Genetics and Genomics, NYU Grossman School of Medicine. Division of Rheumatology, Department of Medicine, NYU Grossman School of Medicine. Department of Biochemistry and Molecular Pharmacology, NYU Grossman School of Medicine, New York, NY, USA, New York, NY, 7UTHealth Houston Division of Rheumatology, Houston

    Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by widespread fibrosis, vasculopathy, autoantibody presence, and high mortality in its diffuse subtype with limited effective…
  • Abstract Number: 1570 • ACR Convergence 2025

    Hydroxychloroquine and Disease Progression in Systemic Sclerosis: Insights from Antibody-Stratified Survival Analyses

    Francesco Bonomi1, Ilaria Bisconti2, Ilenia Mallia3, Greta Pellegrino4, Gabriele Ciuti5, Gloria Muolo2, Martina Salerno2, Simona Truglia2, Cristiano Barbetta3, Silvia Peretti5, Serena Guiducci6, Valeria Riccieri7 and Silvia Bellando Randone3, 1University Hospital Careggi, Florence, Florence, Toscana, Italy, 2Sapienza University of Rome, Rome, Rome, Italy, 3University of Florence, Florence, Italy, 4Rheumatology Unit, IRCCS Ospedale Galeazzi Sant'Ambrogio, Milano, Italy; Department of Biomedical and Clinical Sciences, University of Milan, Milan, Italy, 5University Hospital Careggi, Florence, Italy, 6Division of Rheumatology, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, Florence, Italy, 7Sapienza University of Rome, Rheumatology Clinic, Rome, Italy, Roma, Italy

    Background/Purpose: Hydroxychloroquine (HCQ) is an antimalarial drug that interferes with dendritic cells and monocytes, acidifies endosomes, and modulates toll-like receptors, reducing autoimmune responses and cell…
  • Abstract Number: 1037 • ACR Convergence 2025

    Risk Factor Identification and Dynamic Individualized Prediction of Muscle Involvement in Systemic Sclerosis

    Zixing Liu1, Adrianne Woods2, Julie Paik2, Laura Hummers2, Scott Zeger1, Ami Shah2 and Ji Soo Kim3, 1Johns Hopkins Biostatistics, Baltimore, MD, 2Johns Hopkins Rheumatology, Baltimore, MD, 3Johns Hopkins, Baltimore, MD

    Background/Purpose: Myopathy in SSc significantly increases disability, reduces quality of life, and elevates mortality risk, yet remains understudied. Most identified risk factors derive from cross-sectional…
  • Abstract Number: 0960 • ACR Convergence 2025

    Pharmaceutical Blockade of the Neonatal Fc Gamma Receptor Ameliorates Autoimmunity, Inflammation and Fibrosis in the Topoisomerase I Mouse Model of Systemic Sclerosis

    Cuong Tran-Manh1, Thuong Trinh-Minh2, Christoph Liebel3, Bas van der Woning4, Piotr Zabrocki5 and Jörg Distler6, 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, 4argenx BV, Boston, MA, 5argenx, Ghent, Belgium, 6University Hospital Duesseldorf and HHU, Duesseldorf, Germany

    Background/Purpose: SSc is a rare, chronic, systemic autoimmune rheumatic disease characterized by progressive skin and internal organ fibrosis, severe vasculopathy and immune dysregulation with production…
  • Abstract Number: 0686 • ACR Convergence 2025

    Use of a Smartphone App which Incorporates Feedback to Patients to Monitor Systemic Sclerosis-related Digital Ulcers – a Potential New Tool for Remote Clinical Monitoring

    Ariane Herrick1, Paul New2, Graham Dinsdale3, Andy Vail2, Joanne Manning3, Michael Hughes4, Will dixon5, Chris Taylor5, Mark Dickinson5 and Andrea Murray6, 1The University of Manchester, UK, Aberdeen, United Kingdom, 2The University of Manchester, Manchester, United Kingdom, 3Northern Care Alliance NHS Foundation Trust, Salford, United Kingdom, 4Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK, Manchester, England, United Kingdom, 5The University of Manchester, Manchester, 6University of Manchester, Salford, United Kingdom

    Background/Purpose: To facilitate clinical trials we previously developed a smartphone app to allow ‘tracking’ of systemic sclerosis (SSc)-related digital ulcers (DUs) and their associated pain…
  • Abstract Number: 0882 • ACR Convergence 2025

    Serum KL-6 as a Predictive Biomarker for Interstitial Lung Disease Progression and Mortality in Systemic Sclerosis: A Prospective Cohort Study

    cristiana Sieiro1, Jose Ordas Martínez2, Sara Calleja Antolín2, Juan Garcia Herrero2, Javier De la Calle2, Miriam Retuerto2, Laura Sierra2, Elena Bollo de Miguel2 and Elvira Díez Álvarez2, 1Univrsity of Manchester, Manchester, United Kingdom, 2Complejo Asistencial Universitario de León, León, Spain

    Background/Purpose: Interstitial lung disease (ILD) is a leading cause of morbidity and mortality in systemic sclerosis (SSc). Early detection and continuous monitoring of ILD are…
  • Abstract Number: 0002 • ACR Convergence 2025

    KITE-363: An Autologous Anti-CD19/CD20 CAR-T Product for the Treatment of Autoimmune Rheumatic Diseases

    Brian Kim, Christine Lowe, Francisco Flores, Jeremy Margaitis, Alessandro Calo, Stacey Valny, Anna Konecny, Eva Jaghatspanyan, Sean Yoder, Kenneth Ertel, Simone Filosto, Jodi Murakami and David Barrett, Kite, a Gilead Company, Santa Monica, CA

    Background/Purpose: B-cell dysregulation is a key factor in the development and progression of autoimmune diseases, and B-cell inhibition has been a cornerstone of treatment for…
  • Abstract Number: 2496 • ACR Convergence 2025

    Comparing Long-term Outcome Across Systemic Sclerosis Subgroups Using a Multi-Organ Disease Progression Score

    Philip Yee1, medha kanitkar2, stefano rodolfi3, Voon H. Ong2 and Christopher Denton4, 1Division of Medicine, Centre for Rheumatology, University College London, London, England, United Kingdom, 2Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, 3University College London, London, United Kingdom, 4University College London, UK, London, United Kingdom

    Background/Purpose: Evaluating disease modification in systemic sclerosis (SSc) is challenging and requires long term studies. This is especially relevant to limited cutaneous (lc)SSc where severe…
  • Abstract Number: 2472 • ACR Convergence 2025

    Digesting the data: tracking gastro-intestinal manifestations in systemic sclerosis over time

    Cosimo Bruni1, Jasmin Klöti2, Aurora Maria Tatu3, Lea Stamm2, Rucsandra Dobrota4, Muriel Elhai5, Mike Becker6, Sinziana Muraru7, Gesa Sauer8, Anna-Maria Hoffmann-Vold9, Oliver Distler10 and Carina Mihai11, 1University of Zurich, Zurich, Switzerland, 2Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 3Department of Gastroenterology, University Hospital Zurich, University of Zurich, 8091, Switzerland, 4Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zurich, Switzerland, 5University Hospital zurich, Zürich, Switzerland, 6Dept. of Rheumatology, University Hospital Zurich, Zürich, Switzerland, 7University of Zurich, University Hospital Zurich, Zürich, Switzerland, 8Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, zurich, Switzerland, 9Oslo University Hospital, Oslo, Norway, 10Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland, 11University Hospital Zurich, University of Zurich, Zurich, Switzerland

    Background/Purpose: Gastrointestinal (GI) involvement is among the most frequent organ manifestations in systemic sclerosis (SSc), yet the timing of presentation of GI manifestations remains incompletely…
  • Abstract Number: 1856 • ACR Convergence 2025

    Spatial Proteomic-based Phenotyping of Fibroblast Populations and their Microenvironment in Systemic Sclerosis Primary Heart Involvement

    Ayla Nadja Stuetz1, Giacomo de Luca2, Alexandru-Emil Matei3, Yi-Nan Li4, Veronica Batani2, Tim Filla5, Aleix Rius Rigau6, Bilgesu Safak Tümerdem1, Cosimo Bruni7, Maike Büttner-Herold8, Stefania Rizzo9, Monica De Gaspari9, Markus Eckstein10, Georg Schett11, Cristina Basso9, Jörg Distler12, Marco Matucci-Cerinic13 and Andrea-Hermina Györfi14, 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, 2Vita-Salute San Raffaele University. Unit of Immunology, Rheumatology, Allergy and Rare Diseases, IRCCS San Raffaele Hospital, Milan, Italy, 3Department 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, 4University Hospital of Düsseldorf, Düsseldorf, Germany, 5Department 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, 6Department of Internal Medicine 3, Rheumatology and Clinical Immunology, Friedrich-Alexander-University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen. Deutsches Zentrum Immuntherapie (DZI), Friedrich-Alexander University (FAU) Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 7Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 8Department of Nephropathology, University Hospital Erlangen, Erlangen, Germany, 9Cardiovascular Pathology Unit, Department of Cardiac, Thoracic and Vascular Sciences, University and Hospital of Padua, Padua, Italy, 10Institute of Pathology and Comprehensive Cancer Center EMN, Friedrich-Alexander- Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, 11Uniklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg, Germany, Erlangen, Germany, 12University Hospital Duesseldorf and HHU, Duesseldorf, Germany, 13University San Raffaele Milano, Milano, Milan, Italy, 14Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University., Düsseldorf, Germany

    Background/Purpose: Primary heart involvement (pHI) is one of the leading causes of death in systemic sclerosis (SSc). However, the cellular and molecular pathomechanisms of SSc-pHI…
  • Abstract Number: 1568 • ACR Convergence 2025

    A Novel Endothelial Cell Profiling Assay to Identify Systemic Sclerosis Patient Characteristics at the Endothelial Cell Level

    Eva Hoekstra1, Rudmer Postma1, Nils Steinz2, Wieke van Oostveen1, Roel Bijkerk1, Vincent van Duinen2, Rachel Knevel2, Tom Huizinga2, Anton jan van Zonneveld1, Jeska de Vries-Bouwstra2 and Cynthia Fehres1, 1Leiden University Medical Center, Leiden, Zuid-Holland, Netherlands, 2Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: Vasculopathy, a hallmark clinical feature in systemic sclerosis (SSc), is a consequence of endothelial cell (EC) damage and dysfunction and might precede tissue fibrosis.…
  • Abstract Number: 1015 • ACR Convergence 2025

    Refining Administrative Algorithms For Accurate Identification of Patients with Systemic Sclerosis In Trinetx Research Network

    Gulsen Ozen1, Michael O'Rorke2, Paul Romitti2 and Robyn Domsic3, 1University of Iowa, Carver College of Medicine, Division of Immunology, Coralville, IA, 2University of Iowa College of Public Health, Department of Epidemiology, Iowa City, IA, 3University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Large data-driven medical research is invaluable in answering questions about epidemiology, genetics, therapeutics, and outcomes of rare diseases. Systemic sclerosis (SSc) is rare yet…
  • Abstract Number: 0957 • ACR Convergence 2025

    Multi-Omic Profiling Reveals a Monocyte-Vascular Signature Associated with the Regression of Skin Fibrosis in SSc

    Astrid Hofman1, Pietro Bearzi2, Elena Pachera3, Cosimo Bruni4, Lumeng Li2, Laura Much5, Kristina Bürki1, Mike Becker6, Anna-Maria Hoffmann-Vold7 and Oliver Distler8, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zurich, Switzerland, 2Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, the LOOP Zurich, Zürich, Switzerland, 3University Hospital Zurich, Zurich, Switzerland, 4University of Zurich, Zurich, Switzerland, 5Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 6Dept. of Rheumatology, University Hospital Zurich, Zürich, Switzerland, 7Oslo University Hospital, Oslo, Norway, 8Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland

    Background/Purpose: Regression of skin fibrosis is a feature of the natural history of dcSSc. The molecular mechanisms underlying this resolution remain unclear. This study aims…
  • Abstract Number: 0685 • ACR Convergence 2025

    Targeting Refractory Reflux in Systemic Sclerosis: Early Outcomes following a Novel Intervention

    Areeka Memon1, Monique Hinchcliff2 and Amir Masoud3, 1Yale New Haven Health, New Haven, CT, 2Yale School of Medicine, Westport, CT, 3Hartford Healthcare, hartford

    Background/Purpose: Gastrointestinal tract (GIT) symptoms impact up to 90% of individuals with SSc. Hiatal hernia, decreased esophageal contractility, and lower esophageal sphincter tone contribute to…
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