Abstracts tagged "Systemic sclerosis"

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…
Abstract Number: 0328 • ACR Convergence 2022
Predicting Progression from Undifferentiated to Definitive Connective Tissue Diseases: A Systematic Review and Meta-analysis
Sarah Dyball¹, Mia Rodziewicz¹, Claudia Mendoza Pinto², Ian N. Bruce³ and Ben Parker⁴, ¹The University of Manchester, Manchester, United Kingdom, ²Systemic Autoimmune Diseases Research Unit, High-Specialty Medical Unit-CIBIOR, Mexican Social Security Institute, Puebla, Mexico, ³Centre for Epidemiology Versus Arthritis, Faculty of Biology, Medicine and Health, The University of Manchester, Manchester, United Kingdom, ⁴Manchester University Hospitals NHS Foundation Trust, Manchester, United Kingdom
Background/Purpose: Undifferentiated connective tissue disease (UCTD) encapsulates a broad range of conditions including incomplete forms of systemic lupus erythematosus (SLE) and systemic sclerosis (SSc), some…
Abstract Number: 1061 • ACR Convergence 2022
Safety and Tolerability of Aminaphtone in Systemic Sclerosis Patients: A Four-year Follow-up
andrea cere, Emanuele Gotelli, Lercara Adriano, Carmen Pizzorni, sabrina Paolino, Alessandri Elisa, Maurizio Cutolo and Alberto Sulli, Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS San Martino Polyclinic Hospital, Genova, Italy
Background/Purpose: Recent studies proved that aminaphtone is an effective treatment for Raynaud's phenomenon (RP) secondary to systemic sclerosis (SSc). Laser Speckle Contrast Analysis (LASCA) further…
Abstract Number: 1183 • ACR Convergence 2022
Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq
Kristina Clark¹, Alice Cole², Shiwen Xu¹, Voon Ong³, Christopher Buckley⁴ and Chris Denton¹, ¹University College London, London, United Kingdom, ²Royal Free Hospital, London, United Kingdom, ³UCL Medical School Royal Free Campus, London, United Kingdom, ⁴University of Oxford, Oxford, United Kingdom
Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…
Abstract Number: 1533 • ACR Convergence 2022
Use of Body Mass Index in Prediction of Interstitial Lung Disease Related to Systemic Sclerosis Revealed by Decision Trees
Alexandru Garaiman¹, Carina Mihai¹, Rucsandra Dobrota¹, cosimo bruni², Muriel Elhai¹, Suzana Jordan¹, Lea Stamm³, Anna-Maria Hoffmann-vold⁴, Oliver Distler¹ and Mike Becker¹, ¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²University of Florence, Florence, Italy, ³Department of Rheumatology, University Hospital Zurich, Zürich, Switzerland, ⁴Oslo University Hospital, Oslo, Norway
Background/Purpose: Some gastrointestinal (GI) and nutritional factors are associated with the presence of interstitial lung disease related to systemic sclerosis (SSc-ILD). However, there are many…
Abstract Number: 2158 • ACR Convergence 2022
A Decade of Caring for SSc Patients: Do Outcomes Improve? Data from the Leiden CCISS Cohort
Sophie Liem¹, Saad Ahmed², Jacopo Ciaffi³, Liesbeth Beaart-van de Voorde⁴, Anne Schouffoer⁵, J Geelhoed⁶, Nina Ajmone Marsan⁷, Tom Huizinga⁸ and Jeska de Vries-Bouwstra⁸, ¹LUMC, Leiden, Netherlands, ²Leiden University Medical Centre, Leiden, Netherlands, ³IRCCS Istituto Ortopedico Rizzoli, Bologna/Leiden University Medical Center, Bologna, Italy, ⁴Leiden University Medical Center, Leiden, Netherlands, ⁵Leiden University Medical Center/Haga Teaching Hospital, Leiden, Netherlands, ⁶Leiden University Medical Center, Pulmonology, Leiden, Zuid-Holland, Netherlands, ⁷Leiden University Medical Center, Cardiology, Leiden, Netherlands, ⁸Leiden University Medical Center, Leiden, Netherlands
Background/Purpose: Combined Care in Systemic Sclerosis (CCISS) is a prospective cohort of patients referred to Leiden University Medical Center for Raynaud's Phenomenon (RP), a suspicion…

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