Abstracts tagged "Scleroderma"

Abstract Number: 0957 • ACR Convergence 2024
Increased Collagen Deposition and Altered Immune Cell Profiles Are Present in Early and Late Stage Systemic Sclerosis with Gastrointestinal Involvement
Laura Much¹, Elena Pachera², Andrea Laimbacher¹, Henriette Didriksen³, Lars Aabakken⁴, Knut Ea Lundin⁴, Lumeng Li¹, Astrid Hofman¹, Pietro Bearzi⁵, Sophie Wagner⁶, Michael Scharl⁷, Øyvind Molberg⁸, Håvard Fretheim⁹, Oliver Distler¹⁰ and Anna-Maria Hoffmann-Vold⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Oslo University Hospital, Moss, Norway, ⁴Department of Transplantation Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁶University of Zurich, Schlieren, Switzerland, ⁷Department of Gastroenterology and Hepatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁸Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway, ⁹Oslo University Hospital, Oslo, Norway, ¹⁰Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Gastrointestinal tract (GIT) involvement is highly prevalent among patients with systemic sclerosis (SSc) and is associated with high morbidity and mortality. However, treatment options…
Abstract Number: 1571 • ACR Convergence 2024
Growth Differentiation Factor 15 Is a Serum Biomarker and Pathogenic Factor of Progressive Fibrosis in Systemic Sclerosis
Vishal Kakkar¹, Sunhwa Kim², Yingtao Bi³, Christopher Wasson¹, Stefano Di Donato⁴, Rebecca Ross⁵, Marco Di Battista⁶, Enrico De Lorenzis⁷, Thierry Sornasse² and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²AbbVie, South San Francisco, CA, ³Abbvie, Worcester, MA, ⁴Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁵Medicine and Health, University of Leeds, Leeds, United Kingdom, ⁶Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Pisa, United Kingdom, ⁷Catholic University of the Sacred Heart, Roma, Rome, Italy
Background/Purpose: In systemic sclerosis (SSc), interstitial lung disease (ILD) remains the leading cause of mortality. A decline in forced vital capacity (FVC) is considered a…
Abstract Number: 1824 • ACR Convergence 2024
Skin Macrophage Subtypes and Impact of Tofacitinib in Early Diffuse Cutaneous Systemic Sclerosis: Results from Single-cell Analyses of an Observational Data Set and a Phase I/II Randomized Controlled Trial
Juliette Ferrant¹, Alain Lescoat², Valérie Lecureur³, Marie Lelong³, John Varga⁴, Robert Lafyatis⁵, Johann Gudjonsson⁴ and Dinesh Khanna⁴, ¹CHU Rennes, Rennes, France, ²CHU Rennes - University Rennes 1, Rennes, France, ³Rennes University, Rennes, France, ⁴University of Michigan, Ann Arbor, MI, ⁵University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Macrophages play a major role in dcSSc-related skin fibrosis, with a mixed M1-M2 activation profile relying on the activation of JAK/STAT. Tofacitinib, a pan-JAK…
Abstract Number: 2444 • ACR Convergence 2024
Performance of the DETECT Algorithm and Cluster Analysis in Screening for Systemic Sclerosis Pulmonary Hypertension Groups
Ryan Osgueritchian¹, Hoda Mombeini¹, Vivek Jani², Adrianne Woods³, Steven Hsu¹, Matthew Lammi⁴, Paul Hassoun⁴, Fredrick Wigley⁵, Laura Hummers⁶, Stephen Mathai⁷, Ami Shah⁸ and Monica Mukherjee¹, ¹Johns Hopkins University Division of Cardiology, Baltimore, MD, ²Johns Hopkins University School of Medicine, Baltimore, MD, ³Johns Hopkins University Division of Rheumatology, Baltimore, MD, ⁴Johns Hopkins University Division of Pulmonary and Critical Care Medicine, Baltimore, MD, ⁵Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Baltimore, MD, ⁶Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ⁷Johns Hopkins University Division of Pulmonary and Critical Care Medicine,, Blatimore, ⁸Division of Rheumatology, Johns Hopkins University, Ellicott City, MD
Background/Purpose: Pulmonary hypertension (PH) portends poor outcomes in Systemic Sclerosis (SSc). Recent efforts for early identification and intervention in SSc patients with pulmonary arterial hypertension…
Abstract Number: 2688 • ACR Convergence 2024
HLA-DRB4: A Novel Susceptibility Locus in Systemic Sclerosis Patients with Severe Calcinosis
Sara Faghihi-Kashani¹, srijana davluri², Kamini Kuchinad³, Zuoming deng⁴, Faiza Naz⁴, Stefania Dell'Orso⁴, Zsuzsanna McMahan⁵, Laura Hummers⁶, Daniel Kastner⁷, Fredrick Wigley³, david fiorentino⁸, Christian Lood⁹, Ami Shah¹⁰, Lorinda Chung¹¹ and Pravitt Gourh⁴, ¹Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, San Francisco, CA, ²Division of Immunology and Rheumatology, Stanford University School of Medicine, Stanford, CA, Sunnyvale, CA, ³Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Baltimore, MD, ⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, ⁵UTHealth Houston Division of Rheumatology, Houston, TX, ⁶Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ⁷National Human Genome Research Institute, Bethesda, MD, Bethesda, MD, ⁸Department of Dermatology, Stanford University School of Medicine, Stanford, CA, Palo Alto, CA, ⁹Division of Rheumatology, University of Washington, Seattle, WA, Seattle, WA, ¹⁰Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ¹¹Stanford University, Woodside, CA
Background/Purpose: Systemic sclerosis (SSc) is a complex autoimmune disease. Class II HLA alleles have been reported to play an important role in SSc pathogenesis. Calcinosis, deposition of…
Abstract Number: 0693 • ACR Convergence 2024
Behind the Bronchiectasis in Systemic Sclerosis. Prevalence and Risk Factors
Miriam Retuerto Guerrero¹, Clara Moriano², Paula Perez Garcia³, cristiana sieiro santos⁴ and elvira Diez álvarez³, ¹Complejo Asistencial Universitario de León, Leon, Spain, ²Hospital León, LEON, Spain, ³Complejo Asistencial Universitario de Leon, Leon, ⁴Rheumatology Department, Complejo Asistencial Universitario de León, León, Spain, Leon, Spain
Background/Purpose: Bronchiectasis (BC) has been observed in association with Systemic Sclerosis (SSc). Esophageal dysmotility, immunosuppressive drugs and the direct effect of collagen deposition in the…
Abstract Number: 0960 • ACR Convergence 2024
The Esophageal Epithelium in Systemic Sclerosis: Cellular and Molecular Dysregulation Revealed by Single-Cell RNA Sequencing
Matthew Dapas¹, Margarette Clevenger¹, Hadijat Makinde², Tyler Therron¹, Dustin Carlson¹, Mary Carns³, Kathleen Aren³, Carrie Richardson², Cenfu Wei², Lutfiyya Muhammad⁴, John Pandolfino¹, Harris Perlman², Deborah Winter⁵ and Marie-Pier Tetreault¹, ¹Northwestern University, Chicago, ²Northwestern University, Chicago, IL, ³Northwestern University Division of Rheumatology, Chicago, IL, ⁴Northwestern Feinberg School of Medicine, Chicago, IL, ⁵Northwestern University, Skokie, IL
Background/Purpose: Systemic sclerosis (SSc) is a rare autoimmune disease characterized by vasculopathy and progressive fibrosis of the skin and internal organs. Individuals with SSc often…
Abstract Number: 1572 • ACR Convergence 2024
Associations Between the Gut Microbiota, Ultra-Processed Food Intake, and Gastrointestinal Tract Symptoms in Patients with Systemic Sclerosis
Ju Young Lee¹, Swapna Joshi², Arissa Young³, Jen Labus², Zsuzsanna McMahan⁴, Ezinne Aja², Jonathan Jacobs² and Elizabeth Volkmann⁵, ¹David Geffen School of Medicine, UCLA, Los Angeles, CA, ²UCLA, Los Angeles, ³UCLA, Los Angeles, CA, ⁴UTHealth Houston Division of Rheumatology, Houston, TX, ⁵University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Alterations in the gastrointestinal (GI) microbiome (i.e., dysbiosis) are a feature of systemic sclerosis (SSc) [1]. Diet is a known modifier of the GI…
Abstract Number: 1825 • ACR Convergence 2024
Proteomic and Transcriptomic Analysis Reveal Elevated Senescent Signature in Systemic Sclerosis Fibrosis
Poulami Dey, Hiroshi Kato, Suiyuan Huang, Johann Gudjonsson, Dinesh Khanna, John Varga and Eliza Pei-Suen Tsou, University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is marked by persistent fibrosis affecting both the skin and internal organs. Some regard SSc as a manifestation of expedited aging,…
Abstract Number: 2450 • ACR Convergence 2024
Disease Progression in Anti-Centromere Positive Systemic Sclerosis Patients
Eva Hoekstra¹, S. Ahmed², David Ueckert³, Nina Ajmone Marsan³, Philippine Kiès³, M.K. Ninaber³, Marlies Heuvers³, Miranda Geelhoed³, Thomas Huizinga⁴ and Jeska de Vries-Bouwstra¹, ¹Leiden University Medical Center, Leiden, Netherlands, ²Leiden University Medical Centre, Leiden, Netherlands, ³Leids Universitair Medisch Centrum, Leiden, Netherlands, ⁴Department of Rheumatology, Leiden University, Leiden, Netherlands
Background/Purpose: Disease progression in systemic sclerosis (SSc) is characterized by severe and early progression in anti-topoisomerase (ATA) positive diffuse cutaneous patients and typically involves lung…
Abstract Number: PP08 • ACR Convergence 2024
Whispers of Resilience: Navigating Life with Rheumatism
Vinchelle Hardison and Vinchelle Hardison, Nationwide Children's Hospital, Columbus, OH
Background/Purpose: “What’s wrong with me mom? Why am I always slow and hurting? Why can’t I play without being in pain like the other kids?”…
Abstract Number: 0694 • ACR Convergence 2024
A Unified Vascular Phenotype Score Identifies Systemic Sclerosis Endotypes and Predicts Prognostic Outcomes: Results from the EUSTAR Database
Stefano Di Donato¹, Michael Hughes², John Pauling³, Marco Matucci-Cerinic⁴, Lesley-Anne Bissell⁵, Edward Jude⁶, Christopher Denton⁷, Yannick Allanore⁸, Marie-Elise Truchetet⁹ and Francesco Del Galdo¹⁰, and EUSTAR collaborators, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ²Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ³North Bristol NHS Trust, Bristol, United Kingdom, ⁴University San Raffaele Milano, Milano, Milan, Italy, ⁵Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁶Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, United Kingdom, ⁷University College London, Northwood, United Kingdom, ⁸Université Paris Cité, Paris, France, ⁹Bordeaux University Hospital, Bordeaux, France, ¹⁰University of Leeds, Leeds, United Kingdom
Background/Purpose: Vascular dysfunction is pivotal in Systemic Sclerosis (SSc) pathogenesis, leading to somatic vascular signs such as pitting scars, Digital Ulcers (DUs), telangiectasia as well…
Abstract Number: 0964 • ACR Convergence 2024
Engaging the PD-1 Pathway Attenuates Inflammation Associated Fibrosis in Systemic Sclerosis Fibroblasts and a Preclinical Mouse Model
Maithri Aspari¹, Voon Ong², Klaus Soendergaard³, Esben Naeser⁴, Malene Hvid⁴, Angela Tam⁵, Shiwen Xu⁵, Christopher Denton⁶, David Abraham⁷, Bent Deleuran¹ and Stinne Greisen⁸, ¹Aarhus University, Aarhus, Denmark, ²University College London, London, England, United Kingdom, ³Aarhus University Hospital, Aarhus, Denmark, ⁴AARHUS UNIVERSITET, AARHUS C, Denmark, ⁵University College London, London, United Kingdom, ⁶University College London, Northwood, United Kingdom, ⁷UCL, London, United Kingdom, ⁸Aarhus University/Aarhus University Hospital, Aarhus, Denmark
Background/Purpose: The precise molecular mechanisms driving fibrosis in diffuse cutaneous systemic sclerosis (dcSSc) remain to be elucidated. The immune regulatory programmed cell death protein 1…
Abstract Number: 1573 • ACR Convergence 2024
Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures
Iqtidar Hanif¹, Shervin Assassi¹, Maureen Mayes¹, Meng Zhang¹, Julio Charles¹, John VanBuren², Jessica Alvey², Kimia Ghaffari², Elana Bernstein³, Flavia Castelino⁴, Lorinda Chung⁵, Luke Evnin⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Laura Hummers¹⁰, Dinesh Khanna¹¹, Kimberly Lakin¹², Dorota Lebiedz-Odrobina¹³, Yiming Luo¹⁴, Ashima Makol¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Carrie Richardson¹⁸, Nora Sandorfi¹⁹, Ami Shah²⁰, Ankoor Shah²¹, Victoria Shanmugam²², Virginia Steen²³, Elizabeth Volkmann²⁴, Carleigh Zahn¹¹ and Brian Skaug¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²University of Utah, Salt Lake City, UT, ³Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁴Massachusetts General Hospital, Boston, MA, ⁵Stanford University, Woodside, CA, ⁶Scleroderma Research Foundation, Brisbane, CA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹¹University of Michigan, Ann Arbor, MI, ¹²Hospital for Special Surgery, New York, NY, ¹³University of Utah, Cottonwood Heights, UT, ¹⁴Columbia University, New York, NY, ¹⁵Mayo Clinic, Rochester, MN, Rochester, MN, ¹⁶University of Minnesota, Minneapolis, MN, ¹⁷Northwestern Memorial Hospital, Chicago, IL, ¹⁸Northwestern University, Chicago, IL, ¹⁹University of Pennsylvania, Philadelphia, PA, ²⁰Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ²¹Duke University, Durham, NC, ²²Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, ²³Georgetown University School of Medicine, Washington, DC, ²⁴University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Raynaud’s phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc). RP alerts clinicians to the possibility of an autoimmune rheumatic disease,…
Abstract Number: 1826 • ACR Convergence 2024
Unveiling the Primary Cilia Signature Driving Systemic Sclerosis Pathogenesis
Le My Tu Nguyen¹, Carlos Córdova-Fletes², Poulami Dey³, Johann Gudjonsson³, Rebecca Wells⁴, Rebecca Ross⁵, Natalia Riobo-Del Galdo⁴, Francesco Del Galdo⁴, Dibyendu Bhattacharyya³, John Varga³ and Maria Teves¹, ¹Virginia Commonwealth University, Richmond, VA, ²Universidad Autónoma de Nuevo León, Monterrey, Mexico, ³University of Michigan, Ann Arbor, MI, ⁴University of Leeds, Leeds, United Kingdom, ⁵Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Fibrosis in multiple organs is the defining hallmark that accounts for the high mortality associated with systemic sclerosis (SSc). Understanding how SSc patients develop…

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