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Abstracts tagged "Scleroderma"

  • Abstract Number: 0682 • ACR Convergence 2024

    Results from Outcomes Selection and Development of a Patient-reported Outcome Measure for a Combined Response Index for Limited Cutaneous SSc: The CRISTAL Project

    Alain Lescoat1, Yen Chen2, Susan Murphy3, Nadia Vann2, Neda Kortam2, Rosemary Gedert2, Sue Farrington4, Yannick Allanore5, David Cella6, Lorinda Chung7, Philip Clements8, Christopher Denton9, Francesco Del Galdo10, Oliver Distler11, Monique Hinchcliff12, Michael Hughes13, Laura Hummers14, John Pauling15, Janet Pope16, Virginia Steen17, John Varga2, Peter Merkel18, Maya H. Buch19 and Dinesh Khanna2, and all CRISTAL collaborators, 1CHU Rennes - University Rennes 1, Rennes, France, 2University of Michigan, Ann Arbor, MI, 3University of Michigan, Plymouth, MI, 4Scleroderma & Raynaud UK, London, United Kingdom, 5Université Paris Cité, Paris, France, 6Northwestern University, Chicago, IL, 7Stanford University, Woodside, CA, 8United States, Los Angeles, CA, 9University College London, Northwood, United Kingdom, 10University of Leeds, Leeds, United Kingdom, 11Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, 12Yale School of Medicine, Westport, CT, 13Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, 14Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, 15North Bristol NHS Trust, Bristol, United Kingdom, 16University of Western Ontario, London, ON, Canada, 17Georgetown University School of Medicine, Washington, DC, 18University of Pennsylvania, Philadelphia, PA, 19Division of Musculoskeletal & Dermatological Sciences, University of Manchester, and NIHR Manchester Biomedical Research Centre, Manchester, United Kingdom

    Background/Purpose: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of scleroderma, yet there is a paucity of outcome measures available to assess symptoms…
  • Abstract Number: 0785 • ACR Convergence 2024

    Exploring the Link Between Atgl-Dependent Lipolysis and Dermal Fibrosis in Systemic Sclerosis

    Elizabeth Caves1, Agrani Dixit1, Anna Jussila2, Vivian Lei3, Hailey Edelman4, Muhammad Hamdan5, Ian Odell5, Monique Hinchcliff6, Radhika Atit7 and Valerie Horsley1, 1Yale School of Medicine, New Haven, CT, 2Stanford Medicine, Stanford, CA, 3University of North Carolina School of Medicine, Chapel Hill, 4Vanderbilt School of Engineering, New Haven, 5Yale School of Medicine, New Haven, 6Yale School of Medicine, Westport, CT, 7Case Western Reserve University College of Arts and Sciences, Cleveland

    Background/Purpose: Resident lipid-filled dermal adipocytes are depleted in both systemic sclerosis (SSc) and scleroderma mouse models, but mechanisms are poorly understood. We undertook studies in mouse…
  • Abstract Number: 1568 • ACR Convergence 2024

    Distribution of Proteinuria in Scleroderma Renal Crisis: A Multi-Center Study

    Arifa Javed1, Lilit Grigoryan2, Alex Luta3, Sumbal Wajid2, Lee Shapiro4, Virginia Steen5 and Swati Mehta6, 1Albany Medical Center, Rensselaer, NY, 2Albany Medical Center, Albany, NY, 3MedStar Georgetown University Hospital, Rockville, MD, 4Albany Medical College, Stillwater, NY, 5Georgetown University School of Medicine, Washington, DC, 6Division of Nephrology, Department of Medicine, Albany Medical Center, Albany, NY

    Background/Purpose: Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc), characterized by malignant hypertension and acute kidney injury (AKI). Proteinuria when present at the time of…
  • Abstract Number: 1819 • ACR Convergence 2024

    Insulin-like Growth Factor Binding Protein-7 (IGFBP7) Plays a Pathogenic Role in Dermal Fibrosis and Is Increased in Systemic Sclerosis

    Ye Jin Jeong1, Gayathri Guru Murthy1, Gaochan Wang1, Tahia Rahman1 and Benjamin Korman2, 1University of Rochester School of Medicine and Dentistry, Rochester, NY, 2University of Rochester, Rochester, NY

    Background/Purpose: Systemic sclerosis (SSc, scleroderma) is an autoimmune connective tissue disease that causes autoimmunity, vascular dysfunction, and fibrosis of the skin, lungs, and other organs.…
  • Abstract Number: 2052 • ACR Convergence 2024

    Role of Scleroderma/myositis-related Autoantibodies Detected by Immunoblot to the Diagnosis of Systemic Autoimmune Rheumatic Diseases in 410 Patients from a Single Referral Center

    Carmen Secada Gómez1, Diana Prieto-Peña2, Mónica Renuncio-García3, Juan Irure-Ventura4, Marcos Lopez-Hoyos5 and Ricardo Blanco-Alonso6, 1Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, 2Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Cantabria, Spain, 3Division of Immunology, Hospital Universitario Marqués de Valdecilla, Immunopathology Group, IDIVAL, Santander, Spain, 4Division of Immunology, Hospital Universitario Marqués de Valdecilla. Immunopathology group, IDIVAL, Santander, Cantabria, Spain, 5Division of Immunology, Hospital Universitario Marqués de Valdecilla. Immunopathology group, IDIVAL, Santander, Spain, 6Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain

    Background/Purpose: In clinical practice, immunoblot assays are being used more frequently as a diagnostic tool for systemic autoimmune rheumatic diseases (SARDs).Our aim was to evaluate the…
  • Abstract Number: 2644 • ACR Convergence 2024

    Baseline Fibroblast Immunophenotype Predicts Clinical Improvement Among Individuals with Early Diffuse Cutaneous Systemic Sclerosis

    Kimberly Lakin1, Robert Spiera2, John Spivack1, Yaxia Zhang1, Jessica Gordon3 and dana Orange4, 1Hospital for Special Surgery, New York, NY, 2Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, 3Division of Rheumatology, Weill Cornell Medical College, New York, NY, 4Rockefeller University, New York, NY

    Background/Purpose: We have previously shown that fibroblast CD34 is low and alpha-smooth muscle actin (aSMA) is high in severe diffuse cutaneous systemic sclerosis (dcSSc) skin,…
  • Abstract Number: 0689 • ACR Convergence 2024

    Arrhythmic Burden, Myocardial Markers, and Long-term Survival in Distinct Cardiac Magnetic Resonance Subsets of Systemic Sclerosis

    Silvia Laura Bosello1, Enrico De Lorenzis2, Giacomo De Luca3, Antonio Tonutti4, veronica Batani5, Pier Giacomo Cerasuolo6, Gerlando Natalello7, Lucia Lanzo6, Gabriella Alonzi6, Silvia Fiore6, Stefano Di Murro6, Andrea Zoli6, Valentina Boni6, Riccardo Marano8, Francesca Augusta Gabrielli9, Francesco Del Galdo10, Dan Knight11, Vivek Muthurangu11, Christopher Denton12, Maria De Santis4, Marco Matucci Cerinic5 and Maria Antonietta D'Agostino13, 1Unit of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, 2Catholic University of the Sacred Heart, Roma, Rome, Italy, 3Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan, Milan, Italy, 4Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy, 5Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, 6Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, 7Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, 8Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Diagnostic Imaging Area, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Rome, Italy, 9Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, 10University of Leeds, Leeds, United Kingdom, 11UCL Department of Cardiac MRI, University College London (Royal Free Campus), London, United Kingdom, 12University College London, Northwood, United Kingdom, 13Division of Rheumatology, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Università Cattolica del Sacro Cuore, Rome, Italy

    Background/Purpose: Cardiac involvement in Systemic Sclerosis (SSc) is widely recognized as heterogeneous and, when clinically evident, it is associated with a poor prognosis. Recently, five…
  • Abstract Number: 0954 • ACR Convergence 2024

    Increased Proliferating Natural Killer Cells Exhibit an Aberrant Pro-Inflammatory Gene Signature in Systemic Sclerosis Patients

    Pietro Bearzi1, Elena Pachera2, Sophie Wagner3, Astrid Hofman4, Lumeng Li4, Laura Much4, Mike Becker5, Kristina Bürki4, Luca Navarini6, Anna-Maria Hoffmann-Vold7, Roberto Giacomelli8 and Oliver Distler9, 1Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, 2University Hospital Zurich, Zurich, Switzerland, 3University of Zurich, Schlieren, Switzerland, 4Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 5Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 6Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Rome, Italy, 7Oslo University Hospital, Oslo, Norway, 8Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Roma, Italy, 9Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland

    Background/Purpose: Recent transcriptomic data suggest a prominent involvement of innate immune cells in the pathogenesis of SSc. In this regard, contrasting data on NK cells…
  • Abstract Number: 1570 • ACR Convergence 2024

    Deconvolution of the Lipidomic Signature of Very Early Diagnosis of Systemic Sclerosis (VEDOSS) and Established Disease: Lipid Biomarker Features That Predict Disease Progression in Skin and Lung

    Sunhwa Kim1, Yingtao Bi2, Vishal Kakkar3, Rebecca Ross4 and Francesco Del Galdo3, 1AbbVie, South San Francisco, CA, 2Abbvie, Worcester, MA, 3University of Leeds, Leeds, United Kingdom, 4Medicine and Health, University of Leeds, Leeds, United Kingdom

    Background/Purpose: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease with variable clinical presentations and progression rates. Accurate patient stratification is crucial for optimizing treatment strategies.…
  • Abstract Number: 1822 • ACR Convergence 2024

    Analysis of Differential Activation of Lysophosphatidic Acid Regulated Genes in Diffuse and Limited Cutaneous Systemic Sclerosis

    Medha Kanitkar1, Philip Yee2, Stefano Rodolfi1, Kristina Clark3, Voon Ong4 and Christopher Denton5, 1University College London, London, United Kingdom, 2UCL, London, United Kingdom, 3University of Oxford, Oxford, United Kingdom, 4University College London, London, England, United Kingdom, 5University College London, Northwood, United Kingdom

    Background/Purpose: Lysophosphatidic Acid (LPA) is a lipid mediator implicated in the pathogenesis of SSc and  idiopathic pulmonary fibrosis. Phase 2 clinical trials targeting this biological…
  • Abstract Number: 2441 • ACR Convergence 2024

    Investigating Kidney Disease Risks in Systemic Sclerosis (SSc) – Effects of Proton Pump Inhibitors (PPI): Retrospective Analysis

    Irakli Tskhakaia and Arthur Lau, Jefferson Einstein Hospital, Philadelphia, PA

    Background/Purpose: Systemic Sclerosis (SSc) patients often experience gastrointestinal complications, frequently managed with proton pump inhibitors (PPIs). Despite evidence of PPI-related chronic kidney disease (CKD) risk in…
  • Abstract Number: 2684 • ACR Convergence 2024

    Assessment of Skin in Patients with Systemic Sclerosis Using High Frequency Ultrasound : A Comparative Study with Histology and Clinical Parameters of Skin Disease

    Ruhani Desai1, Filemon Tan2, Minghua Wu3, Jefferey Browning4, Samuel Theodore3, Meng Zhang3, Brian Skaug3, Maureen Mayes3 and Shervin Assassi3, 1UTHealth Houston Division of Rheumatology, DeLand, FL, 2University of Texas at Houston McGovern Medical School, Houston, TX, 3UTHealth Houston Division of Rheumatology, Houston, TX, 4Boston Univeristy, Boston, MA

    Background/Purpose: The extent of skin involvement and its rate of progression can provide prognostic information for systemic sclerosis (SSc)-related morbidity and mortality. Moreover, skin assessment…
  • Abstract Number: 0630 • ACR Convergence 2023

    Prevalence and Risk Factors for Systemic Sclerosis Digital Ischemic Complications in the Collaborative National Quality and Efficacy Registry

    Marissa Savoie1, Monica Harding2, Jessica Alvey2, John VanBuren2, Shervin Assassi3, Elana Bernstein4, Lorinda Chung5, Luke Evnin6, Tracy Frech7, Jessica Gordon8, Faye Hant9, Laura Hummers10, Dinesh Khanna11, Kimberly Lakin8, Dorota Lebiedz-Odrobina12, Yiming Luo13, Ashima Makol14, Jerry Molitor15, Duncan Moore16, Carrie Richardson17, Nora Sandorfi18, Ami Shah19, Ankoor Shah20, Brian Skaug21, Virginia Steen22, Elizabeth Volkmann23 and Flavia Castelino1, 1Massachusetts General Hospital, Boston, MA, 2University of Utah, Salt Lake City, UT, 3University of Texas McGovern Medical School at Houston, Houston, TX, 4Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, 5Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, 6Scleroderma Research Foundation, Brisbane, CA, 7Vanderbilt University Medical Center, Nashville, TN, 8Hospital for Special Surgery, New York, NY, 9Medical University of South Carolina, Charleston, SC, 10Johns Hopkins University, Baltimore, MD, 11University of Michigan, Ann Arbor, MI, 12University of Utah, Cottonwood Heights, UT, 13Division of Rheumatology, Department of Medicine, Columbia University Irving Medical Center, New York, NY, 14Mayo Clinic, Rochester, MN, Rochester, MN, 15University of Minnesota, Minneapolis, MN, 16Northwestern University, Department of Rheumatology, Chicago, IL, 17Northwestern University, Chicago, IL, 18University of Pennsylvania, Philadelphia, PA, 19Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, 20Duke University, Durham, NC, 21Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, 22Georgetown University School of Medicine, Washington, DC, 23University of California Los Angeles, Los Angeles, CA

    Background/Purpose: Digital ischemic ulcers (DIU) develop in 36-44% of patients with systemic sclerosis (SSc).1,2Prior international observational studies have evaluated characteristics of digital pitting and ulcers…
  • Abstract Number: 0792 • ACR Convergence 2023

    Single Cell RNA-seq of Myeloid Cells from Systemic Sclerosis Patients Identifies Circulating Monocyte Population with Interferon Signature Associated with Interstitial Lung Disease

    Richard Ainsworth1, Kim Taylor2, Ye Cao3, Takanori Sasaki4, Deepak Rao5, Nunzio Bottini6 and Francesco Boin1, 1Cedars-Sinai Medical Center, Los Angeles, CA, 2University of California San Francisco, San Francisco, CA, 3Brigham and Women's Hospital, Boston, MA, 4Brigham and Women's Hospital and Harvard Medical School, Boston, MA, 5Brigham and Women's Hospital, Boston, MA, 6University of California, San Francisco, CA

    Background/Purpose: Growing evidence supports a critical role for innate immunity in systemic sclerosis (SSc) pathogenesis. Altered myeloid cell numbers and functions have been implicated in…
  • Abstract Number: 1262 • ACR Convergence 2023

    “Somebody Who’s Been There and Can Understand the Challenges That You’re Going Through”: Participant Perspectives of a Resilience-Based Energy Management Online Intervention for Systemic Sclerosis with Peer Health Coaches

    Yen Chen1, Alexandra Harper2, Tiffany Phanhdone3, Mary Alore1, Sheri Hicks1, Adam Pape1, Gina Jay1, Shannen Bolde1, Jennie Feldpausch1, Dinesh Khanna1 and Susan Murphy4, 1University of Michigan, Ann Arbor, MI, 2University of Michigan, Ypsilanti Charter Twp, MI, 3Northwestern University, Chicago, IL, 4University of Michigan, Plymouth, MI

    Background/Purpose: People with systemic sclerosis (SSc) often experience fatigue, which impacts daily life functioning and quality of life. We developed a 12-week, resilience-based energy management…
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