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Abstracts tagged "Fibrosing syndromes"

  • Abstract Number: 1872 • ACR Convergence 2025

    High-throughput Screening Uncovers Distinct Molecular Signatures Linked with HRCT Patterns in SARD-ILD

    Philip Stauffer1, Vyacheslav Palchevskiy2, Laura Much3, Phuong Diep4, Magdalena Abel4, Natasha Moe4, Thomas Gaisl5, Carolin Steinack5, Shahrzad Lari2, Elena Pachera6, Øyvind Molberg7, Oliver Distler8, S. Samuel Weigt2, John A. Belperio2 and Anna-Maria Hoffmann-Vold4, 1University of Zurich Hospital, Schlieren, Switzerland, 2UCLA, Los Angeles, 3Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 4Oslo University Hospital, Oslo, Norway, 5USZ, Zürich, Switzerland, 6University Hospital Zurich, Zurich, Switzerland, 7Department of Rheumatology, Oslo University Hospital, Oslo, Norway, 8Department of Rheumatology, University Hospital Zurich, University of Zurich, Switzerland, Zurich, Switzerland

    Background/Purpose: High-Resolution Computed Tomography (HRCT) is crucial for diagnosing and managing interstitial lung disease associated with systemic autoimmune rheumatic diseases (SARD-ILD), as it reveals characteristic…
  • 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: 1163 • ACR Convergence 2025

    Clinical and Biomarker Characteristics of Inebilizumab-Treated Participants Who Experienced an IgG4-RD Flare During the Phase 3 MITIGATE Trial

    John Stone1, Emma Culver2, Arezou Khosroshahi3, Wen Zhang4, Emanuel Della Torre5, Kazuichi Okazaki6, Yoshiya Tanaka7, Matthias Lohr8, nicolas schleinitz9, Fernando Martinez-Valle10, Manu Nayar11, Vinciane Rebours12, Cory Perugino13, Kristen Clarkson14, Theresa Alexander14, Xinxin Dong14, Qian Huang14, Sue Cheng14 and Daniel Cimbora14, 1Massachusetts General Hospital , Harvard Medical School, Concord, MA, 2John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom, 3Emory University, Atlanta, GA, 4Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 5IRCCS San Raffaele Scientific Institute, Milan, Italy, 6Kansai Medical University Kori Hospital, Osaka, Japan, 7University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, 8Karolinska Institutet, Stockholm, Sweden, 9Aix Marseille university, AP-HM, Marseille, France, 10Vall d’Hebron Hospital, Barcelona, Spain, 11Freeman Hospital, Newcastle upon Tyne, United Kingdom, 12Beaujon Hospital, AP-HP, Université Paris-Cité, Clichy, France, 13Massachusetts General Hospital, Harvard Medical School, Boston, MA, 14Amgen, Thousand Oaks, CA

    Background/Purpose: IgG4-RD is a rare, chronic, fibroinflammatory disorder characterized by recurrent flares that can affect any organ and lead to permanent tissue damage. Inebilizumab (INEB)…
  • 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: 0968 • ACR Convergence 2025

    Mesenchymal Stromal Cells in Systemic Sclerosis are Dysfunctional and Have a Profibrotic and Senescent Phenotype

    Marianela Brizio1, Benoit Brilland1, Maximilien Lora2, Mathieu Mancini1, David Langlais1, Marie Hudson3 and Ines Colmegna4, 1Research Institute of the McGill University Health Centre, Montreal, QC, Canada, 2Research Institute of the McGill University Health Centre, Montreal, 3McGill University, Montréal, QC, Canada, 4The Research Institute of the McGill University Health Centre, Montréal, QC, Canada

    Background/Purpose: Mesenchymal stromal cells (MSCs) are non-hematopoietic multipotent cells with immunomodulatory, proangiogenic, and antifibrotic properties. MSC functions are mediated by paracrine soluble factors and small…
  • Abstract Number: 0966 • ACR Convergence 2025

    Integrated Bulk and Single Cell Analysis Confirms Differential Upregulation of the Proton Sensing Receptor GPR68 in Systemic Sclerosis Across Disease Stage and Subset

    kristina clark1, Xu Shiwen2, Xue Li2, Voon H. Ong3 and Christopher Denton4, 1Kennedy Institute of Rheumatology, University of Oxford, London, United Kingdom, 2University College London, London, United Kingdom, 3Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, 4University College London, UK, London, United Kingdom

    Background/Purpose: The cell surface protein GPR68 (ovarian cancer G protein-coupled receptor 1, OGR1) is a proton-sensing G-protein-coupled receptor (GPCR) that responds to extracellular acidity and…
  • Abstract Number: 0958 • ACR Convergence 2025

    Asynchronous Resolution of Inflammation and Fibrosis in A Prolonged Experimental Model Suggests Distinct Temporal Dynamics And Resolution Mechanisms in Systemic Sclerosis

    Aurore Collet1, Manel Jendoubi2, Thomas Guerrier2, Alexis Largy2, Silvia Speca2, Sylvain Dubucquoi1 and David Launay3, 1Univ. Lille, Inserm, CHU Lille, U1286 – INFINITE – Institute for Translational Research in Inflammation, Lille, France ; CHU Lille, Institut d’Immunologie, Lille, France, Lille, France, 2Univ. Lille, Inserm, CHU Lille, U1286 – INFINITE – Institute for Translational Research in Inflammation, Lille, France, Lille, France, 3Univ. 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

    Background/Purpose: Systemic sclerosis (SSc) is characterized by tissue fibrosis, which is defined as excessive and irreversible extracellular matrix (ECM) accumulation, leading to organ dysfunction. In…
  • Abstract Number: 0692 • ACR Convergence 2025

    A Randomised Open Label Pilot Trial Comparing Mycophenolate Mofetil with no Immunosuppression in Limited Cutaneous Systemic Sclerosis (MINIMISE-Pilot)

    Christopher Denton1, Philip Yee2, medha kanitkar3, Charlotte Clarke4, Saiam Ahmed4, Voon H. Ong3, Francesco Del Galdo5, John Pauling6, Marina Anderson7, Muditha Samaranayaka8, Michael Hughes9, Smita Bhat10, Bridget Griffiths11, MAYA BUCH12, David D'Cruz13, Ariane Herrick14, Madelon Vonk15, Nicholas Feemantle4 and Dehbi Hakim-Moulay4, 1University College London, UK, London, United Kingdom, 2Division of Medicine, Centre for Rheumatology, University College London, London, England, United Kingdom, 3Division of Medicine, Centre for Rheumatology, University College London, London, United Kingdom, 4University College London, London, United Kingdom, 5University of Leeds, Leeds, United Kingdom, 6North Bristol NHS Trust, Bristol, United Kingdom, 7Lancaster University and NHS University Hospitals of Liverpool Group, Liverpool, United Kingdom, 8Salford Royal Hospital, Salford, United Kingdom, 9Division of Musculoskeletal and Dermatological Sciences, The University of Manchester, Manchester Academic Health Science Centre, Manchester, UK, Manchester, England, United Kingdom, 10Ninewells Hospital Dundee, Dundee, Scotland, United Kingdom, 11Ninewells Hospital Dundee, Newcastle, England, United Kingdom, 12UNIVERSITY OF MANCHESTER, MANCHESTER, United Kingdom, 13Kings College London, London, United Kingdom, 14The University of Manchester, UK, Aberdeen, United Kingdom, 15Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands

    Background/Purpose: Mycophenolate mofetil (MMF) is recommended for skin in diffuse cutaneous (dc)SSc, and for lung fibrosis in SSc, but patients with limited cutaneous (lc)SSc are…
  • Abstract Number: 0279 • ACR Convergence 2025

    Assessment of Baseline IgG4-RD Disease Characteristics and Impact Upon Safety and Efficacy of Inebilizumab: Results from the MITIGATE Study

    Yoshiya Tanaka1, Emma Culver2, Arezou Khosroshahi3, Wen Zhang4, Kazuichi Okazaki5, Matthias Lohr6, nicolas schleinitz7, Xinxin Dong8, melissa rosen9, Sue Cheng8, Daniel Cimbora8 and John Stone10, 1University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, 2John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom, 3Emory University, Atlanta, GA, 4Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 5Kansai Medical University Kori Hospital, Osaka, Japan, 6Karolinska Institutet, Stockholm, Sweden, 7Aix Marseille university, AP-HM, Marseille, France, 8Amgen, Thousand Oaks, CA, 9Amgen Inc., Thousand Oaks, CA, 10Massachusetts General Hospital , Harvard Medical School, Concord, MA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a progressive, systemic, fibroinflammatory disease characterized by unpredictable and recurring flares, leading to organ damage and decreased quality of life.…
  • Abstract Number: 0099 • ACR Convergence 2025

    Time course and impact of  IL17A on hepatic inflammation and fibrosis in adjuvant induced arthritis

    Arthur Dollinger1, Frédéric Meyer1, Hélène Martin2, Céline Delougeot3, Delphine Weil-Verhoeven1 and Frank Verhoeven4, 1CHU Besancon, Besancon, France, 2Université Marie et Louis Pasteur, Besancon, France, 3université Marie et Louis Pasteur, Besancon, 4CHU de Besançon, Besançon, Franche-Comte, France

    Background/Purpose: The management of spondyloarthritis. (SpA) includes controlling disease activity as well as comorbidities such as metabolic syndrome. Metabolic syndrome can be associated with metabolic…
  • Abstract Number: 0038 • ACR Convergence 2025

    DNA Methylation Signatures of Smoking in Labial Salivary Gland Tissue in a Sjögren’s Disease Cohort

    Priya Bhatt1, Mary Horton2, Caroline Shiboski3, Lisa Barcellos4 and Lindsey Criswell2, 1Mayo Clinic Alix School of Medicine, Toledo, 2NIH/NHGRI, Bethesda, MD, 3University of California San Francisco, San Francisco, CA, 4UC Berkeley, Berkeley

    Background/Purpose: Cigarette smoking has been linked to the development of several autoimmune diseases, including Sjögren’s Disease (SjD). Since DNA methylation (DNAm) is altered by cigarette…
  • Abstract Number: 2476 • ACR Convergence 2025

    Blinatumomab in rapid progressive systemic sclerosis

    Christina Gebhardt1, Franziska Szelinski2, Hector Rincon-Arevalo2, Giulia Magno3, Veit Buecklein3, Gerulf Haenel4, Gerhard Zugmaier5, Michael von Bergwelt3, Marion Subklewe3, Thomas Dörner6, Alla Skapenko7 and Hendrik Schulze-Koops7, 1LMU Hospital, Division for Rheumatology and Clinical Immunology, München, Germany, 2Charite Universitétsmedizin Berlin, Germany, Berlin, Germany, 3LMU Klinikum, Med. Klinik und Poliklinik III, Munich, Germany, 4LMU Gene Center, Munich, Germany, 5Amgen, Munich, Germany, 6Charite Universitétsmedizin Berlin, Germany and DRFZ, Berlin, Berlin, Germany, 7LMU Hospital, Division for Rheumatology and Clinical Immunology, Munich, Bayern, Germany

    Background/Purpose: Systemic sclerosis is a severe, potentially fatal disease, characterized by progressive fibrosis of skin and internal organs. Blinatumomab is a bispecific CD3/CD19-T-cell engager (BiTe)…
  • Abstract Number: 2020 • ACR Convergence 2025

    Inebilizumab Efficacy and Safety in Patients with Common, Urgent, and Fibrotic Organ Manifestations of IgG4-RD: Subgroup Analyses from the MITIGATE Trial

    Arezou Khosroshahi1, Emma Culver2, Wen Zhang3, Kazuichi Okazaki4, Yoshiya Tanaka5, Matthias Lohr6, nicolas schleinitz7, Xinxin Dong8, Sue Cheng8, Daniel Cimbora8 and John Stone9, 1Emory University, Atlanta, GA, 2John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom, 3Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 4Kansai Medical University Kori Hospital, Osaka, Japan, 5University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, 6Karolinska Institutet, Stockholm, Sweden, 7Aix Marseille university, AP-HM, Marseille, France, 8Amgen, Thousand Oaks, CA, 9Massachusetts General Hospital , Harvard Medical School, Concord, MA

    Background/Purpose: IgG4-related disease (IgG4-RD) is a progressive, systemic, fibroinflammatory disease characterized by unpredictable and recurring flares, leading to organ damage and decreased quality of life.…
  • Abstract Number: 2019 • ACR Convergence 2025

    Incidence of Coronavirus Disease 2019 in Participants with IgG4-Related Disease Treated with Inebilizumab in the MITIGATE Study

    Arezou Khosroshahi1, Emma Culver2, Wen Zhang3, Kazuichi Okazaki4, Yoshiya Tanaka5, Matthias Lohr6, nicolas schleinitz7, Xinxin Dong8, Sue Cheng8, Daniel Cimbora8 and John Stone9, 1Emory University, Atlanta, GA, 2John Radcliffe Hospital; University of Oxford, Oxford, United Kingdom, 3Peking Union Medical College Hospital, Dong Cheng Qu, China (People's Republic), 4Kansai Medical University Kori Hospital, Osaka, Japan, 5University of Occupational and Environmental Health, Japan, Kitakyushu, Japan, 6Karolinska Institutet, Stockholm, Sweden, 7Aix Marseille university, AP-HM, Marseille, France, 8Amgen, Thousand Oaks, CA, 9Massachusetts General Hospital , Harvard Medical School, Concord, MA

    Background/Purpose: The potential of B-cell-depleting therapies to diminish humoral responses is recognized, but whether this leads to an increased risk of Coronavirus Disease 2019 (COVID-19)…
  • Abstract Number: 1876 • ACR Convergence 2025

    Targeting the NK cell checkpoint NKG2A promotes lung fibrosis resolution by enhancing immune clearance of senescent myofibroblasts

    Wolfgang Merkt1, Lea Rodon2, Franca Sophie Deicher3, Maren Claus4, Rachel Lister5, Hongwei Han5, Yan Zhou5, Zhengwang Sun5, Arik Horne6, Ayla Nadja Stuetz7, Michael Kreuter8, nicolas kahn9, Marc Schneider9, Simon Haas10, Norbert Blank11, Hanns-Martin Lorenz12, Carsten Watzl4, Daniel Hübschmann13 and David Lagares5, 1University Hospital Düsseldorf, Düsseldorf, Germany, 2University Hospital Heidelberg, Heidelberg, Hungary, 3Uniklinik Düsseldorf, Düsseldorf, Germany, 4IfADo, Leibniz Institute Dortmund, Dortmund, Germany, 5Massachusetts General Hospital and Harvard Medical School, Boston, 6Charite, Berlin, Berlin, 7Department 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, 8Universitätsmedizin Mainz, Mainz, 9Thoraxklinik, Heidelberg University, Heidelberg, Germany, 10Charite, Berlin, Berlin, Germany, 11University Hospital Heidelberg, Heidelberg, Germany, 12Universitétsklinikum Heidelberg, Heidelberg, Germany, 13Heidelberg University, Berlin, Germany

    Background/Purpose: A key event driving pulmonary fibrosis in interstitial lung disease (ILD) is the accumulation of pathologic senescent myofibroblasts, thought to be promoted by insufficient…
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