Abstracts tagged "Systemic sclerosis"

Abstract Number: 1874 • ACR Convergence 2022
Autoantibodies Are Common in Patients with Idiopathic Interstitial Lung Disease Suggesting a High Prevalence of Undiagnosed Autoimmune Connective Tissue Disease
Sarah Tansley¹, Caroline Cotton², Fionnuala McMorrow¹, Hui Lu¹, Aravinthan Loganathan¹, Zoe Betteridge¹, Robert New³, Lisa Spencer⁴, Neil McHugh¹ and Robert Cooper⁴, ¹University of Bath, Bath, United Kingdom, ²Liverpool University Hospital, Liverpool, United Kingdom, ³NCA, Manchester, ⁴University of Liverpool, Liverpool
Background/Purpose: In some patients, interstitial lung disease (ILD) may be the dominant or even sole overt manifestation of an otherwise unrecognised autoimmune connective tissue disease…
Abstract Number: 1051 • ACR Convergence 2022
A Deep Neural Network Classifier to Identify Intrinsic Molecular Subsets of Systemic Sclerosis Using Histological Images
Yiwei Yuan¹, Qingyuan Song¹, Paul Harms², Johann Gudjonsson², Robert Lafyatis³, Dinesh Khanna⁴ and Michael Whitfield⁵, ¹Dartmouth College, Lebanon, NH, ²University of Michigan, Ann Arbor, MI, ³Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ⁴Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ⁵Geisel School of Medicine, Lebanon, NH
Background/Purpose: Intrinsic gene analyses based on the gene expression profiles in skin have identified four intrinsic molecular subsets among patients with systemic sclerosis (SSc); these…
Abstract Number: 1180 • ACR Convergence 2022
Deficiency and Altered Phenotype of Mucosal-associated Invariant T Cells in Systemic Sclerosis
Manon Lesturgie-Talarek¹, Virginie Gonzalez¹, Camelia Frantz², Noémie Sénot¹, Zouriatou Gouda¹, Camille Rousseau¹, Lucie Beaudoin¹, Agnès Lehuen¹, Jérôme Avouac³ and Yannick Allanore⁴, ¹INSERM U1016, Institut Cochin, Paris, France, ²Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ³University of Paris, Paris, France, ⁴Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of the skin and internal organs such as the lung. Mucosal-associated invariant T (MAIT)…
Abstract Number: 1527 • ACR Convergence 2022
Maintained Complete B Cell Depletion Is Associated with Rituximab Efficacy in Connective Tissue Disorder Interstitial Lung Disease
Jérôme Avouac¹, Roba Ghossan², Omar Al Tabaa³, Alice Combier², Alexia Steelandt², Marion Thomas⁴ and Yannick Allanore⁵, ¹University of Paris, Paris, France, ²Hôpital Cochin, AP-HP.Centre - Université Paris Cité, Paris, France, ³Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ⁴Hôpital Cochin, AP-HP Centre - Université Paris Cité, Paris, France, ⁵Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: The benefit of RTX has been suggested for interstitial lung disease (ILD) associated with connective tissue disorders (CTD). Preliminary data have suggested that B…
Abstract Number: 1885 • ACR Convergence 2022
Histopathological Patterns of Muscle Involvement and Clinical Correlations in a Retrospective Cohort of Scleroderma Patients
Noemie Le Gouellec¹, Louai Zaidan², Eric Hachulla³, Nicolas Dognon³, Robin Dhote⁴, Sebastien Riviere⁵, Yurdagul Uzunhan⁴, Luc Mouthon⁶ and Jerome Authier⁷, ¹CH Valenciennes, Valenciennes, France, ²CHU Henri Mondor, Creteil, France, ³University of Lille, LILLE, France, ⁴Hopital Avicenne - Paris University, Bobigny, France, ⁵Hopital Saint Antoine - Paris University, Paris, France, ⁶Hopital Cochin - Paris University, Paris, France, ⁷Paris Est - Creteil University, Creteil, France
Background/Purpose: Muscle involvement in systemic sclerosis (SSc) has been shown to be heterogeneous. Histopathological pattern seems to be associated with prognosis, but studies evaluating the…
Abstract Number: 0154 • ACR Convergence 2022
Anti-SMN Autoantibodies in Mixed Connective Tissue Disease Are Associated with a Scleromyositis Phenotype
Hajar El Kamouni¹, Alexandra Albert², Sabrina Hoa³, Darya S. Jalaledin⁴, Josiane Bourre-Tessier⁵, Eric Rich⁵, Jean-Richard Goulet⁶, Martial Koenig⁷, Minoru Satoh⁸, Marvin Fritzler⁹, May Choi¹⁰, Yves Troyanov¹¹, Jean-Luc Senécal⁵ and Océane Landon-Cardinal⁵, ¹Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Laval, Canada, ²Division of Rheumatology, Centre Hospitalier de l'Université Laval, Québec, QC, Canada, ³University of Montreal, Montréal, QC, Canada, ⁴Division of Internal Medicine, Centre Hospitalier de l'Université de Sherbrooke, Saint-Lambert, Canada, ⁵Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁶Division of Rheumatology, Centre Hospitalier de l'Université de Montréal, Saint-Lambert, Canada, ⁷Division of Internal Medicine, Centre Hospitalier de l'Université de Montréal, Montréal, QC, Canada, ⁸Department of Human, Information and Science, University of Occupational Environmental Health, Kitakyusyu Fukuoka, Japan, ⁹University of Calgary, Calgary, AB, Canada, ¹⁰Brigham and Women's Hospital | University of Calgary, Calgary, AB, Canada, ¹¹Division of Rheumatology, Hôpital du Sacré-Coeur de Montréal, Montréal, QC, Canada
Background/Purpose: The survival of motor neuron (SMN) is a ubiquitous macromolecular complex, located in the Cajal body of mammalian nuclei, with an essential role in…
Abstract Number: 1053 • ACR Convergence 2022
The Collaborative National Quality and Efficacy Registry for Scleroderma: Association of Resource Utilization and Gastrointestinal Tract Symptoms in Early Disease
Sarah Luebker¹, Tracy Frech¹, Shervin Assassi², Jessica Gordon³, Elana Bernstein⁴, Virginia Steen⁵, Laura Hummers⁶, Ami Shah⁷, Carrie Richardson⁸, Dinesh Khanna⁹, Flavia Castelino¹⁰, Lorinda Chung¹¹, Faye Hant¹², Vicki Shanmugam¹³, John VanBuren¹⁴, Jessica Alvey¹⁴, Monica Harding¹⁴, Luke Evnin¹⁵ and Nora Sandorfi¹⁶, ¹Vanderbilt University Medical Center, Nashville, TN, ²McGovern Medical School, University of Texas, Houston, TX, ³Hospital for Special Surgery, New York, NY, ⁴Columbia University, New York, NY, ⁵Georgetown University School of Medicine, Washington, DC, ⁶Johns Hopkins Univerisity, Baltimore, MD, ⁷Johns Hopkins Rheumatology, Baltimore, MD, ⁸Northwestern University, Chicago, IL, ⁹Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁰Massachusetts General Hospital, Boston, MA, ¹¹Stanford University, Stanford, CA, ¹²Medical University of South Carolina, Charleston, SC, ¹³George Washington University, Washington, DC, ¹⁴University of Utah, Salt Lake City, UT, ¹⁵Scleroderma Research Foundation, Brisbane, CA, ¹⁶University of Pennsylvania, Philadelphia, PA
Background/Purpose: Health costs and resource utilization are important in chronic disease management. Systemic sclerosis (SSc) is a multi-organ system disease with outcomes that are often…
Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Feiyang Ma¹, Pei-Suen Tsou¹, Grace Hile¹, John Varga¹, J. Michelle Kahlenberg¹, Allison Billi¹, Johann Gudjonsson¹ and Dinesh Khanna², ¹University of Michigan, Ann Arbor, MI, ²Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…
Abstract Number: 1528 • ACR Convergence 2022
Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD) and Organ Damage: Data from the SENSCIS Trial
Janet Pope¹, Susanna Proudman², Wendy Stevens³, Joerg Henes⁴, Rozeta Simonovska⁵, Margarida Alves⁶ and Yannick Allanore⁷, ¹University of Western Ontario, London, ON, Canada, ²Rheumatology Unit, Royal Adelaide Hospital, and Discipline of Medicine, University of Adelaide, Adelaide, Australia, ³Department of Rheumatology, St Vincent’s Hospital, Melbourne, Australia, Melbourne, Australia, ⁴Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ⁵mainanalytics GmbH, Sulzbach (Taunus), Germany, Sulzbach (Taunus), Germany, ⁶Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁷Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France
Background/Purpose: The Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI) was developed to quantify organ damage in patients with SSc. We assessed outcomes in the SENSCIS…
Abstract Number: 2070 • ACR Convergence 2022
Health-Related Quality of Life Across the Spectrum of Connective Tissue Diseases: A Latent Profile Analysis
Sarah Dyball¹, John Reynolds², Hector Chinoy³, Ariane Herrick¹, Sahena Haque⁴, Ellen Bruce⁵, Sophia Naz⁶, Ben Parker⁷ and Ian N. Bruce⁸, ¹The University of Manchester, Manchester, United Kingdom, ²University of Birmingham, Birmingham, United Kingdom, ³The University of Manchester, Sale, United Kingdom, ⁴Manchester Foundation Trust, Manchester, United Kingdom, ⁵Kellgren Centre for Rheumatology, Manchester, United Kingdom, ⁶Pennine Acute Hospitals NHS Trust, Manchester, United Kingdom, ⁷Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom, ⁸Centre for Epidemiology Versus Arthritis, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom
Background/Purpose: Poor health-related quality of life (HR-QoL) is well recognised within patients with connective tissue diseases (CTD). We hypothesised that subgroups of patients across the…
Abstract Number: 0191 • ACR Convergence 2022
Patient-reported Outcomes to Assess Dyspnea in Connective Tissue Diseases, Interstitial Lung Disease and Pulmonary Hypertension, a Systematic Literature Review of Measurement Properties
Jacqueline Lemmers¹, Madelon Vonk² and Els Ende, van den³, ¹Radboud University Medical Centre Nijmegen, Nijmegen, Netherlands, ²Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands, ³Radboudumc, Nijmegen, Netherlands
Background/Purpose: This COnsensus-based Standards for the selection of health Measurement INstruments (COSMIN)-based systematic review aims to identify and summarize the quality of measurement properties of…
Abstract Number: 1057 • ACR Convergence 2022
Usefulness of REVEAL 2.0 Score as a Prognosis Tool of All-Cause Pulmonary Hypertension in Systemic Sclerosis Patients: A Multicentric Study
Ivette Casafont-Solé¹, Laura Calvo², Anne Riveros-Frutos³, Carlos De la Puente Bujidos⁴, Pau Alcubilla⁵, Lourdes Mateo¹ and Ivan Castellví⁶, ¹Hospital Universitari Germans Trias i Pujol, Badalona, Spain, ²Hospital Universitario Ramon y Cajal, Madrid, Spain, ³Hospital Universitari Germans Trias i Pujol, Barcelona, Spain, ⁴Ramon y Cajal University Hospital, Madrid, Spain, ⁵Hospital Clínic de Barcelona, Barcelona, Spain, ⁶Hospital Universitari de la Santa Creu i Sant Pau, Barcelona, Spain
Background/Purpose: Pulmonary arterial hypertension (PAH) leads to a progressive right heart failure and death.PAH is a leading cause of death in Systemic Sclerosis (SSc). To…
Abstract Number: 1182 • ACR Convergence 2022
Evaluation of Efficacy, Immunogenicity, and Toxicity of a Novel Drug Candidate for Scleroderma-associated Interstitial Lung Disease
Galina Bogatkevich, Ilia Atanelishvili, C. Beth Perry and Richard Silver, Medical University of South Carolina, Charleston, SC
Background/Purpose: Interstitial lung disease (ILD) is the major cause of mortality among scleroderma (systemic sclerosis, SSc) patients. Although immunosuppressive agents and several other drugs such…
Abstract Number: 1531 • ACR Convergence 2022
Continued Treatment with Nintedanib in Patients with Systemic Sclerosis-Associated Interstitial Lung Disease (SSc-ILD): Three-Year Data from SENSCIS-ON
Yannick Allanore¹, Madelon Vonk², Oliver Distler³, Arata Azuma⁴, Maureen Mayes⁵, Alexandra James⁶, Veronika Kohlbrenner⁷, Margarida Alves⁸, Dinesh Khanna⁹ and Kristin Highland¹⁰, ¹Department of Rheumatology A, Descartes University, APHP, Cochin Hospital, Paris, France, Paris, France, ²Department of Rheumatology, Radboud University Medical Center, Nijmegen, Netherlands, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁴Department of Pulmonary Medicine and Oncology, Graduate School of Medicine, Nippon Medical School, Tokyo, Japan, ⁵Division of Rheumatology and Clinical Immunogenetics, University of Texas McGovern Medical School, Houston, TX, ⁶Elderbrook solutions GmbH, Bietigheim-Bissingen, Germany, Bietigheim-Bissingen, Germany, ⁷Boehringer Ingelheim Pharmaceuticals, Inc., Ridgefield, Connecticut, USA, Ridgefield, CT, ⁸Boehringer Ingelheim International GmbH, Ingelheim am Rhein, Germany, ⁹Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI, ¹⁰Cleveland Clinic, Cleveland, Ohio, USA, Cleveland, OH
Background/Purpose: In the randomized placebo-controlled SENSCIS trial in patients with SSc-ILD, nintedanib reduced the rate of decline in forced vital capacity (FVC) (mL/year) over 52…
Abstract Number: 2157 • ACR Convergence 2022
Evaluation of Functional Disability over Time in the Leiden Systemic Sclerosis (CCISS) Cohort
Sophie Liem¹, Saad Ahmed², Tom Huizinga³, Thea Vliet Vlieland⁴, Sytske Anne Bergstra¹ and Jeska de Vries-Bouwstra³, ¹LUMC, Leiden, Netherlands, ²Leiden University Medical Centre, Leiden, Netherlands, ³Leiden University Medical Center, Leiden, Netherlands, ⁴Department of Orthopaedics, Rehabilitation and Physical Therapy, Leiden University Medical Center, Leiden, Netherlands
Background/Purpose: Functional disability in patients with systemic sclerosis (SSc) greatly influences quality of life. In SSc, the goal of treatment is to decrease disease burden…

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