ACR Meeting Abstracts

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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 0657 • ACR Convergence 2023

    The Value of the Six-Minute Walk Test in Detecting Cardiopulmonary Involvement in Patients with Systemic Sclerosis

    Saad Ahmed, Sophie Liem, Jeska de Vries-Bouwstra and Thomas Huizinga, Leiden University Medical Center, Leiden, Netherlands

    Background/Purpose: Cardiopulmonary involvement (CPI) in systemic sclerosis (SSc) is associated with significant morbidity and mortality. Early detection and timely treatment is warranted. The Six-minute walk…
  • Abstract Number: 0791 • ACR Convergence 2023

    Expression of TL1A, Inflammatory, and Fibrotic Pathways in Patients with Diffuse Systemic Sclerosis

    Heather Llewellyn1, Lam (Alex) Tsoi2, Minghua Wu3, Kristina Grigaityte1, Tony (Yong) Wang1, James R. Seibold1, Ernesto Muñoz-Elias1, Dinesh Khanna4 and Johann E. Gudjonsson4, 1Prometheus Biosciences, Inc., San Diego, CA, 2Department of Dermatology and Biostatistics, University of Michigan, Ann Arbor, MI, 3Department of Internal Medicine, Division of Rheumatology, The University of Texas Health Science Center at Houston, Houston, TX, 4University of Michigan, Ann Arbor, MI

    Background/Purpose: Systemic sclerosis (SSc) is a multiorgan disease characterized by systemic vascular injury, inflammation, and fibrosis. While SSc mainly affects the skin, pulmonary manifestations such…
  • Abstract Number: 0953 • ACR Convergence 2023

    Flavin-containing Monooxygenase (FMO3) Links the Gut and Fibrosis in Systemic Sclerosis

    Priyanka Verma1, Bharath Yalavarthi1, Karen Ho2, Lutfiyya Muhammad3, Seokjo Kim4, Johann Gudjonsson1, Rebecca C Schugar5, Mark Brown6, Xinmin Li6, Stanley L Hazen7, Swati Bhattacharyya1 and John Varga1, 1University of Michigan, Ann Arbor, MI, 2Northwestern University, Chicago, IL, 3Northwestern University Feinberg School of Medicine, Chicago, IL, 44SCM Lifescience Co. Ltd, South Korea, Japan, 5Stanford University, Stanford, CA, 6Department of Cardiovascular & Metabolic Sciences, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, 7Division of Endocrinology, Department of Medicine, Stanford University, Stanford, CA

    Background/Purpose: Trimethylamine (TMA) generated by the gut microbiome is converted into trimethylamine N-oxide (TMAO) via the host enzyme FMO3. The TMA-FMO3-TMAO metaorganismal axis has been…
  • Abstract Number: 1520 • ACR Convergence 2023

    Identifying Core Domains for Clinical Trials in Systemic Sclerosis-Associated Raynaud’s Phenomenon and Digital Ulcers Using the Delphi Consensus Method

    Susanna Proudman1, Michael Hughes2, Nancy Maltez3, Edith Brown4, Virginia Hickey5, shawna grosskleg6, B Shea6, Ariane Herrick7, John Pauling8 and Peter Merkel9, 1Royal Adelaide Hospital and University of Adelaide, Adelaide, Australia, 2Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, United Kingdom, 3Department of Medicine, Division of Rheumatology, The Ottawa Hospital, Ottawa, ON, Canada, 4OMERACT, Manchester, United Kingdom, 5OMERACT, Adelaide, Australia, 6OMERACT, Ottawa, ON, Canada, 7University of Manchester, Salford, United Kingdom, 8North Bristol NHS Trust, Bristol, United Kingdom, 9University of Pennsylvania, Philadelphia, PA

    Background/Purpose: The OMERACT Scleroderma Vascular Disease Working Group sought to identify essential core outcome domains for inclusion in clinical trials focusing on Raynaud's phenomenon (RP)…
  • Abstract Number: 1701 • ACR Convergence 2023

    Treatment Regimens and Mortality in Systemic Sclerosis-associated Pulmonary Arterial Hypertension in Light of the 2022 ESC/ERS Guidelines

    Hilde Jenssen Bjørkekjær1, Cosimo Bruni2, Cathrine Brunborg3, Patricia Carreira4, Paolo Airò5, Carmen Pilar Simeon-Aznar6, Marie-Elise Truchetet7, Alessandro Giollo8, Alexandra Balbir-Gurman9, Mickael Martin10, Christopher Denton11, Armando Gabrielli12, Havard Fretheim13, Imon Barua13, Helle Bitter14, Oyvind Midtvedt13, Torhild Garen15, Kaspar Broch16, Arne Andreassen17, Yoshiya Tanaka18, Gabriela Riemekasten19, Ulf Müller-Ladner20, marco Matucci Cerinic21, Iván Castellvi22, Elise Siegert23, Eric Hachulla24, Oliver Distler2 and Anna-Maria Hoffmann-Vold13, 1Department of Rheumatology, Hospital of Southern Norway, Kristiansand & University of Oslo, Institute of Clinical Medicine, Oslo, Norway, 2Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 3Oslo Centre for Biostatistics and Epidemiology, Research Support Services, Oslo University Hospital - Rikshospitalet, Oslo, Norway, 4Hospital Universitario 12 de Octubre, Madrid, Spain, 5Spedali Civili di Brescia, Scleroderma UNIT, UOC Reumatologia ed Immunologia Clinica, Piazzale Spedali Civili 1, 25123, Brescia, Italy, 6Department of Internal Medicine, Systemic Autoimmune Diseases Unit, Hospital Universitario Vall d'Hebronh, Barcelona, Spain, 7Bordeaux University Hospital, Bordeaux, France, 8University of Verona, Rheumatology Section, Department of Medicine, Verona, Italy, Verona, Italy, 9Rheumatology Institute, Rambam Health Care Campus and Rappaport Faculty of |Medicine, Technion, Haifa, Israel, 10Department of Internal Medicine, INSERM U1313, Poitiers University, Poitiers University Hospital, Poitiers, France, 11University College London, London, United Kingdom, 12Ospedali Riuniti Marche, Ancona, Italy, 13Oslo University Hospital, Oslo, Norway, 14Sorlandet sykehus, Kristiansand, Norway, 15Dept of Rheumatology, University Hospital Oslo, Oslo, Norway, 16Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, KG Jebsen center, Institute for Experimental Medical Research, University of Oslo, Oslo, Norway, 17Oslo University Hospital, Rikshospitalet, Department of Cardiology, Oslo, Norway, 18University of Occupational and Environmental Health, Kitakyushu, Japan, 19University Clinic Schleswit-Holstein (UKSH), Lübeck, Germany, 20Justus Liebig University Gießen, Campus Kerckhoff, Bad Nauheim, Germany, 21Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, 22Hospital de Santa Creu i Sant Pau, Barcelona, Spain, 23Department of Rheumatology, Charité University Hospital, Charité Platz 1, D-10117, Berlin, Germany, 24University of Lille, Lille, France

    Background/Purpose: The 2022 ESC/ERS Guidelines recommend upfront combination therapy for low- and intermediate-risk, and triple therapy for high-risk patients with systemic sclerosis (SSc)-associated pulmonary arterial…
  • Abstract Number: 2365 • ACR Convergence 2023

    Decoding the Peripheral Immune Landscape of Systemic Sclerosis to Investigate Disease Stages and Interstitial Lung Disease Progression

    Vasuki Ranjani Chellamuthu1, Maria Noviani2, Ahmad bin Mohamed Lajam1, Andrea Hsiu Ling Low2 and Salvatore Albani1, 1Translational Immunology Institute, SingHealth, Singapore, Singapore, 2Department of Rheumatology and Immunology, Singapore General Hospital, Singapore, Singapore

    Background/Purpose: Systemic sclerosis (SSc) is a heterogenous autoimmune disease characterized by fibrosis, vascular abnormalities and immune dysregulation. Characterization of peripheral blood immune signatures in relation…
  • Abstract Number: 0618 • ACR Convergence 2023

    Incident versus Prevalent Interstitial Lung Disease in Systemic Sclerosis in the EUSTAR Database: Different Disease Phenotypes and Prognosis

    liubov Petelytska1, Arthiha Velauthapillai2, Lorenzo Tofani3, Patricia Carreira4, Giovanna Cuomo5, Eric Hachulla6, Ivan Castellvi7, Radim Becvar8, Alexandra Balbir-Gurman9, Paolo Airò10, Irena Litinsky11, Lesley Ann Saketkoo12, Madelon Vonk13, Jeska de Vries-Bouwstra14, Anna Maria Hoffmann-Vold15, marco Matucci Cerinic16, Oliver Distler17 and Cosimo Bruni17, 1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 2Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands, 3Department of Statistics, Computer Science, Applications, University of Florence, Florence, Italy, 4Hospital Universitario 12 de Octubre, Madrid, Spain, 5Department of Precision Medicine, “Luigi Vanvitelli” University of Campania, Naples, Italy, 6University of Lille, Lille, France, 7Rheumatology, Hospital Universitari de la Santa Creu i Sant Pau, Sant Just Desvern, Spain, 8Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Praha, Czech Republic, 9Rheumatology Institute, Rambam Health Care Campus and Rappaport Faculty of |Medicine, Technion, Haifa, Israel, 10Spedali Civili di Brescia, Scleroderma UNIT, UOC Reumatologia ed Immunologia Clinica, Piazzale Spedali Civili 1, 25123, Brescia, Italy, 11Tel Aviv Sourasky Medical Center, Tel Aviv, Israel, 12University Medical Center - Comprehensive Pulmonary Hypertension Center and ILD Clinic Programs // New Orleans Scleroderma and Sarcoidosis Patient Care & Research Centeris, New Orleans, LA, 13Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, 14Leiden University Medical Center, Leiden, Netherlands, 15Oslo University Hospital, Department of Rheumatology, Oslo, Norway, 16Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, 17Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

    Background/Purpose: Although 50% of patients with systemic sclerosis (SSc) present with interstitial lung disease (ILD) at baseline, new onset of ILD can also occur later…
  • Abstract Number: 0636 • ACR Convergence 2023

    Chasing Pain: Investigating Somatosensory Profiles in Patients with Rheumatological Diseases Using Quantitative Sensory Testing

    Claus Juergen Bauer1, Ruth Sophie Schrapper2, Simon Petzinna3, Charlotte Behning4, Tim T.A. Bender5, Peter Brossart1, Martin Muecke6 and Valentin Sebastian Schäfer1, 1Clinic of Internal Medicine III, Department of Oncology, Hematology, Rheumatology and Clinical Immunology, University Hospital of Bonn, Bonn, Germany, 2University of Bonn, Bonn, Germany, 3Clinic of Internal Medicine III, Oncology, Hematology, Rheumatology and Clinical Immunology, University Hospital Bonn, Bonn, Germany, 4Institute of Medical Biometry, Informatics and Epidemiology, University Hospital of Bonn, Bonn, Germany, 5University Hospital of Bonn, Bonn, Germany, 6Universital Hospital of Aachen, Aachen, Germany

    Background/Purpose: Pain is a prominent symptom in numerous rheumatological diseases. In order to allow for a standardized quantification and the assessment of nociceptive and non-nociceptive…
  • Abstract Number: 0658 • ACR Convergence 2023

    Evaluating the Associations Between Autonomic Dysfunction, Clinical Phenotype and Gastrointestinal Transit in Patients with Systemic Sclerosis

    Maria Paula Alvarez Hernandez1, Brittany Adler2, Jamie Perin3, Michael Hughes4 and Zsuzsanna McMahan5, 1Hospital Clinico San Carlos, Madrid, Spain, 2Johns Hopkins University, Baltimore, MD, 3Johns Hopkins University School of Medicine, Baltimore, MD, 4Northern Care Alliance NHS Foundation Trust, Salford Care Organisation, Salford, United Kingdom, 5Johns Hopkins Rheumatology, Lutherville, MD

    Background/Purpose: The gastrointestinal (GI) tract is the second most commonly impacted organ in systemic sclerosis (SSc). However, the pathogenesis and clinical expression of GI involvement…
  • Abstract Number: 0827 • ACR Convergence 2023

    Development and Initial Validation of a Brief Measure of Uncertainty in Rheumatic Disease

    Caleb Bolden1, Claire Cook1, Lucy Finkelstein-Fox1, Xiaoqing Fu1, Flavia Castelino1, Hyon K. Choi2, Cory Perugino1, John Stone3, Elyse Park1, Zachary Wallace4 and Daniel Hall1, 1Massachusetts General Hospital, Boston, MA, 2Massachusetts General Hospital and Harvard Medical School, Lexington, MA, 3Massachusetts General Hospital Rheumatology Unit, Harvard Medical School, Concord, MA, 4Massachusetts General Hospital, Newton, MA

    Background/Purpose: Patients with systemic autoimmune rheumatic disease (SARD) are often tasked with monitoring ambiguous and unpredictable physical symptoms on their own. Higher levels of uncertainty…
  • Abstract Number: 0954 • ACR Convergence 2023

    Non-canonical WNTA Promotes Cytoskeletal Rearrangement and Integrin Alpha V Clustering via JNK and ROCK to Control the Activation of Latent TGFβ

    Thuong Trinh-Minh1, Chih-Wei Chen2, Cuong Tran Manh1, yi-nan Li1, Honglin Zhu3, Debomita Chakraborty4, Yun Zhang1, Simon Rauber5, Clara Dees5, Christina Bergmann6, Alexander Kreuter7, Christiane Reuter8, Florian Groeber-Becker8, Beate Eckes9, Oliver Distler10, Andreas Ramming11, Georg Schett12 and Joerg Distler1, 1Clinic 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, Germany, 23 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, 33 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany. 5 Department of Rheumatology, Xiangya Hospital, Central South University, Changsha, China, 43 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 53 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, 6Department Internal Medicine, University Hospital Erlangen, Germany, Erlangen, Germany, 7Clinic for Dermatology, Venereology and Allergology, HELIOS St. Elisabeth Clinic, Oberhausen, Germany, 8Translational Center for Regenerative Therapies, Fraunhofer Institute for Silicate Research (ISC) Würzburg, Würzburg, Germany, 9Translational Matrix Biology, University of Cologne, Cologne, Germany. 9 Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, Köln, Germany, 10Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 11Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 12Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disorder with vasculopathy, inflammation, and fibrosis of the skin and organs. Fibrosis is caused by the abnormal…
  • Abstract Number: 1522 • ACR Convergence 2023

    Treatment of Pulmonary Arterial Hypertension in Patients with Connective Tissue Diseases: A Meta-analysis

    Mustafa Erdogan1, Sinem Nihal Esatoglu2, Burcak Kilickiran Avci3 and Gulen Hatemi2, 1Marmara University - Pendik Training and Research Hospital, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 2Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 3Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Department of Cardiology, Istanbul, Turkey

    Background/Purpose: Treatment options for pulmonary arterial hypertension (PAH) have expanded in the last two decades. However, evidence for the treatment of connective tissue disease-associated PAH…
  • Abstract Number: 1704 • ACR Convergence 2023

    Esophageal Mucosal Erosions Can Predict the Deterioration of Lung Function over a Four-year Follow-up Period and Long-term Mortality in Patients with Interstitial Lung Disease Associated with Scleroderma

    gerlando Natalello1, enrico De Lorenzis1, Ludovica Berardini2, lucrezia verardi1, pier giacomo Cerasuolo1, Alfredo Papa3, Italo De Vitis3, Francesco Varone2, Luca Richeldi2, Maria Antonietta D'Agostino1 and Silvia Bosello1, 1Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy, 2Division of Pulmonology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy, 3Division of Gastroenterology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Italy

    Background/Purpose: Interstitial lung disease (ILD) is a major cause of morbidity and disease-related death in systemic sclerosis (SSc). Esophageal disease is common in SSc, and…
  • Abstract Number: 2366 • ACR Convergence 2023

    Spatial Frequency Domain Imaging as a Novel Method to Quantify Longitudinal Skin Changes in Scleroderma

    Hung Vo1, Aarohi Mehendale2, Anahita Pilvar3, Eugene Kissin4, Marcin Trojanowski5, Michael York6, Darren Roblyer2 and Andreea Bujor6, 1Boston Medical Center, Peabody, MA, 2Department of Biomedical Engineering, Boston University, Boston, MA, 3Department of Electrical and Computer Engineering, Boston University, Boston, MA, 4Boston University, Newton, MA, 5Boston University School of Medicine, Boston, MA, 6Boston University, Boston, MA

    Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by excessive collagen deposition in the skin and internal organs, along with vascular dysfunction. The modified…
  • Abstract Number: 0619 • ACR Convergence 2023

    Different Definitions of Disease Severity, Progression and Outcomes in Systemic Sclerosis Associated Interstitial Lung Disease: A Systematic Literature Review

    liubov Petelytska1, Francesco Bonomi2, Carlo Cannistrà3, elisa Fiorentini4, Silvia Peretti3, Sara Torracchi3, Pamela Bernardini3, Carmela Coccia3, Riccardo De Luca3, Alessio Economou3, Juela Levani5, marco Matucci Cerinic6, Oliver Distler7 and Cosimo Bruni7, 1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 2University of Florence, Firenze, Italy, 3University of Florence, Florence, Italy, 4Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Firenze, Italy, 5Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Florence, Italy, 6Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy, 7Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland

    Background/Purpose: There is no established consensus on defining the clinical course and the outcomes of systemic sclerosis-associated interstitial lung disease (SSc-ILD), both among experts and…
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