Abstracts tagged "Systemic sclerosis"

Abstract Number: 0953 • ACR Convergence 2024
Establishment of Systemic Sclerosis Associated Pulmonary Arterial Hypertension Specific Endothelial Cells Through iPSCs and Their Functional and Molecular Analyses
Yuki Kudo¹, Masaru Kato¹, Yuhei Shibata², Ryo Hisada¹, Michihito Kono¹, Yuichiro Fujieda¹, Olga Amengual³ and Tatsuya Atsumi¹, ¹Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan, Sapporo, Japan, ²Rheumatology Department, Hokkaido Medical Center for Rheumatic Diseases, Sapporo, Japan., Sapporo, Japan, ³Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan
Background/Purpose: Systemic sclerosis associated pulmonary arterial hypertension (SSc-PAH) is of particularly clinical interest since its outcomes remain unfavorable despite modern PAH therapies. Previous reports showed…
Abstract Number: 1424 • ACR Convergence 2024
Associations of Human Leukocyte Antigens (HLA) Class II with Interstitial Lung Disease (ILD) in Patients with Systemic Autoimmune Rheumatic Diseases (SARDs). A Prospective Study in Sequential Patients with SARD-ILD
Panagiotis Panagopoulos¹, Loukas chatzis², Vasiliki Kitsiou³, Katerina Tarassi³, Eirini Chatzinikita⁴, Katerina Malagari⁵, Theodoros Vassilakopoulos⁴, Alexandra Tsirogianni³, Andreas Goules⁶ and Athanasios Tzioufas⁷, ¹Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, ²Pathophysiology Department, Athens School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Cholargos Athens, Greece, ³Immunology and Histocompatibility Department, Evangelismos General Hospital, Athens, Greece, ⁴Department of Physiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, ⁵2nd Department of Radiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, ⁶GENERAL HOSPITAL LAIKO ATHENS, Athens, Greece, ⁷LAIKO HOSPITAL, Athens, Greece
Background/Purpose: Genetic studies in various systemic autoimmune rheumatic diseases (SARDs) support that human leukocyte antigen (HLA) class II alleles are associated with specific autoimmune disorders…
Abstract Number: 1579 • ACR Convergence 2024
Exploring the Cardiovascular Complications of Pulmonary Arterial Hypertension in Individuals with Systemic Sclerosis
Omar Mourad¹, Saif Abu Baker¹, Georges Khattar², Jennifer Jdaidani³, Khalil El-Gharib³ and Ahmad Mustafa³, ¹Staten Island University Hospital- Northwell Health, Staten Island, NY, ²Staten Island University Hospital, Staten Island, NY, ³Staten Island University Hospital- Northwell Health, Staten Island
Background/Purpose: Systemic sclerosis (SS) is a complex autoimmune rheumatic disease characterized by immune-mediated activation of both innate and adaptive immunity. Pulmonary involvement, specifically pulmonary arterial…
Abstract Number: 1820 • ACR Convergence 2024
Disruption of Ca2+ Homeostasis and Enhanced Ca2+ Signaling in Scleroderma Fibroblasts Is Due to the Vicious Cycle Formed by Mitochondrial-derived Reactive Oxygen Species, CaMKII, and ER Extrusion Pump RYR2
Yuzo Ikari, William Brodie, Megan Mattichak, Sirapa Vichaikul, Qi Wu, Dinesh Khanna and Eliza Pei-Suen Tsou, University of Michigan, Ann Arbor, MI
Background/Purpose: Prior research has suggested that manipulating intracellular Ca2+ concentrations might yield anti-fibrotic benefits across various experimental models. Moreover, inhibiting downstream calcium-binding proteins, such as…
Abstract Number: 2203 • ACR Convergence 2024
The Association of Autoantibodies with Clinical Manifestations and Long-term Outcomes in Juvenile- and Adult-onset Systemic Sclerosis
Hideaki Tsuji¹, Ryosuke Hiwa¹, Mirei Shirakashi², Shuji Akizuki³, Ran Nakashima¹, Akira Onishi⁴, Hajime Yoshifuji¹, Masao Tanaka⁴ and Akio Morinobu⁵, ¹Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, Japan, ²Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto, ³Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine, Kyoto City, Japan, ⁴Department of Advanced Medicine for Rheumatic Diseases, Kyoto University Graduate School of Medicine, Kyoto, Japan, ⁵Department of Rheumatology and Clinical Immunology, Graduate School of Medicine, Kyoto University, Kyoto, Japan., Kyoto, Japan
Background/Purpose: Our aim is to explore whether clinical features and long-term outcomes depend on autoantibodies in juvenile- and adult-onset systemic sclerosis (SSc), since they were…
Abstract Number: 2460 • ACR Convergence 2024
Severe Gastrointestinal Involvement in Systemic Sclerosis: A New “Transversal” Subset of Disease to Be Prioritised for Intervention Trials
Marco Minerba¹, Riccardo Bixio², Seda Colak³, Stefano Di Donato⁴, Lesley-Anne Bissell⁵ and Francesco Del Galdo⁶, ¹1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, United Kingdom, ²University of Verona, Verona, Verona, Italy, ³Gulhane Training and Research Hospital, Ankara, Turkey, ⁴Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁵Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁶University of Leeds, Leeds, United Kingdom
Background/Purpose: Gastrointestinal (GI) involvement is the most frequent internal complication of systemic sclerosis (SSc) and, when severe, it has a major influence on patient function.…
Abstract Number: 0698 • ACR Convergence 2024
Association of Systemic Sclerosis with ANCA-Associated Vasculitis and Large Vessel Vasculitis: Impact of Pulmonary Arterial Hypertension
Brett Dinner¹, Ahmed Abdelmaksoud², Ann Igoe³, Taylor Viggiano⁴ and Vivek Nagaraja⁵, ¹Creighton University, Paradise Valley, AZ, ²University of California, Riverside, Riverside, CA, ³Flow, Tempe, AZ, ⁴Mayo Clinic, Phoenix, ⁵Mayo Clinic Arizona, Scottsdale, AZ
Background/Purpose: Systemic sclerosis (SSc) is an autoimmune disorder with excessive fibrosis and vasculopathy. While typically non-inflammatory, frank vasculitis can complicate SSc. Concomitant SSc with ANCA-associated…
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: 1557 • ACR Convergence 2024
Estimation of Cardiovascular Risk in Patients with Systemic Sclerosis – Accuracy of Tools Based on SCORE and Its Modifications Compared to Ultrasound Examination of Subclinical Atherosclerosis
Sabína Oreská¹, Aneta Prokopcová², Hana Storkanova¹, Jaroslav Kudlicka³, Vladimir Tuka⁴, Ondrej Mikes⁴, Zdislava Krupickova⁴, Martin Satny⁴, Eva Chytilova⁴, Jan Kvasnicka⁴, Maja Spiritovic⁵, Barbora Hermankova⁶, Petr Cesak⁷, Marian Rybar⁸, Karel Pavelka⁹, Ladislav Šenolt², Radim Becvar¹, Jiří Vencovský², Michal Vrablik⁴ and Michal Tomcik¹, ¹Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ²Institute of Rheumatology and Department of Rheumatology, First Faculty of Medicine, Charles University, Prague, Czech Republic, ³Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, Czech Republic, ⁴3rd Department of Internal Medicine, General University Hospital and 1st Faculty of Medicine, Charles University, Prague, Czech Republic, Prague, ⁵Institute of Rheumatology, Prague, Czech Republic, Prague, ⁶Institute of Rheumatology, Prague, Czech Republic, Prague, Czech Republic, ⁷Department of Human Movement Laboratory, Faculty of Physical Education and Sport, Charles University, Prague, Czech Republic, Prague, ⁸Department of Biomedical Technology, Faculty of Biomedical Engineering, Czech Technical University in Prague, Kladno, Czech Republic, Kladno, ⁹Institute of Rheumatology and Charles University, Praha, Czech Republic
Background/Purpose: Patients with systemic sclerosis (SSc) have increased cardiovascular (CV) risk due to accelerated atherosclerosis (ATS) caused by systemic inflammation, and vascular impairment. To date,…
Abstract Number: 1581 • ACR Convergence 2024
Neutrophil-to- lymphocyte Ratio: A Possible Biomarker for Clinical Response After Autologous Hematopoietic Stem Cell Transplantation
shiri keret¹, Lisa Kaly², Georg Schett³, Christina Bergmann⁴, Joerg Henes⁵, Gleb Slobodin² and Doron Rimar², ¹Rheumatology unit, Bnai-Zion medical center and the faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Atlit, Israel, ²Rheumatology unit, Bnai-Zion medical center and the faculty of Medicine, Technion - Israel Institute of Technology, Haifa, Israel., Haifa, Israel, ³Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ⁴Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁵University Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Baseline high Neutrophil- to- lymphocyte ratio (NLR) (higher than 2.95) is associated with severe progressive skin and lung disease and with reduced 5-year survival…
Abstract Number: 1821 • ACR Convergence 2024
Single-Cell RNA SequencingBioinformatic Pipeline Optimized for Non-Coding GenesIdentified LINC01503 and MIR193-BHG as Potential Pathogenic Effectors in Systemic Sclerosis Fibroblasts
Sophie Wagner¹, Elena Pachera², Gino Andrea Bonazza³, Celina Geiss⁴, Astrid Hofman⁴, Laura Much⁴, Lumeng Li⁴, Pietro Bearzi³, Anna-Maria Hoffmann-Vold⁵ and Oliver Distler⁶, ¹University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Oslo University Hospital, Oslo, Norway, ⁶Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Long non-coding RNAs (lncRNA) are a class of transcripts that regulate gene expression. Currently, only few lncRNAs have been linked to SSc pathogenesis. As…
Abstract Number: 2204 • ACR Convergence 2024
Guidance for Stem Cell Therapy for Juvenile Systemic Sclerosis Patients
Ivan Foeldvari¹, Kathryn Torok², Juliana Silva³, Paulina Horvei⁴, Christopher Denton⁵, Franziksa Rosser⁶, Tamas Constantin⁷, Patricia Costa Reis⁸, Megan Curran⁹, Maurizio Cutolo¹⁰, Joerg Henes¹¹, Bernd Hinrichs¹², Kim Fligelstone¹³, Suzanne Li¹⁴, Susan Maillard³, Pia Moinzadeh¹⁵, Catherine Orteu¹⁶, Clare Pain¹⁷, Clarissa Pilkington¹⁸, Linda Schraven¹⁹ and vanessa smith²⁰, ¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ²Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ³Great Ormond Street Hospital, London, United Kingdom, ⁴Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵University College London, Northwood, United Kingdom, ⁶Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁷Semmelweis University, Budapest, Hungary, ⁸Hospital de Santa Maria, Lisbon, Portugal, ⁹Children's Hospital Colorado, Colorado, ¹⁰Laboratory of Experimental Rheumatology and Academic Division of Clinical Rheumatology, Department of Internal Medicine, University of Genova, IRCCS Ospedale Policlinico San Martino, Genoa, Italy, Genova, Italy, ¹¹University Hospital Tuebingen, Tuebingen, Germany, ¹²Children's pulmonology, Asklepios Klinik Nord – Heidberg, Hamburg, Germany, ¹³FESCA, London, United Kingdom, ¹⁴Hackensack University Medical Center, Hackensack, ¹⁵University Hospital Cologne, Cologne, Germany, ¹⁶Royal Free London NHS Foundation Trust, London, United Kingdom, ¹⁷Alder Hey Children’s Foundation NHS Trust, Liverpool, United Kingdom, ¹⁸Great Ormond Street Hospital, London, United Kingdom, ¹⁹Dutch representative of Fesca, London, United Kingdom, ²⁰Ghent University Hospital, Gent, Belgium
Background/Purpose: Hemopoetic stem cell transplantation(HSTC) and cellular therapies(CT) are a promising therapeutic options for juvenile systemic sclerosis(jSSc) patients. As pediatric patients have a unique potential…
Abstract Number: 2465 • ACR Convergence 2024
Subclinical Loss of Lung Volumes in Very Early SSc: Evidence from Two Independent Cohorts in EUSTAR
Vishal Kakkar¹, Stefano Di Donato², Silvia Bellando-Randone³, Riccardo Bixio⁴, Marco Matucci-Cerinic⁵, Yannick Allanore⁶ and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ³University of Florence, Florence, Florence, Italy, ⁴University of Verona, Verona, Verona, Italy, ⁵University San Raffaele Milano, Milano, Milan, Italy, ⁶Université Paris Cité, Paris, France
Background/Purpose: Interstitial lung disease (ILD) remains as the major driver of mortality in systemic sclerosis (SSc). The literature indicates that at the time of diagnosis…
Abstract Number: 0699 • ACR Convergence 2024
Cardiac Biomarkers for Predicting Cardiopulmonary Toxicity of Autologous Haematopoietic Stem Cell Transplantation for Systemic Sclerosis
Ross Penglase¹, Samadhi Gunawardhana¹, Graham Jones¹, Andrew Jabbour¹, Eugene Kotlyar¹, Laila Girgis², David Ma¹ and John Moore¹, ¹St Vincent's Hospital Sydney, Darlinghurst, New South Wales, Australia, ²St Vincent's Hospital, Sydney, Darlinghurst, New South Wales, Australia
Background/Purpose: Autologous Haematopoietic Stem Cell Transplantation (AHSCT) is an effective treatment for severe systemic sclerosis (SSc), with demonstrated benefit for pulmonary and cutaneous outcomes and…
Abstract Number: 0955 • ACR Convergence 2024
Inhibition of Interleukin-2-Inducible T Cell Kinase with Soquelitinib Demonstrates Efficacy in Preventing Lung Damage in Murine Models of Systemic Sclerosis
Goncalo Boleto¹, Anne Cauvet², James Rose³, Lih-Yun Hsu³, Richard A. Miller³ and Yannick Allanore⁴, ¹Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal., Lisbon, Portugal, ²Université de Paris, Institut Cochin, INSERM U1016 CNRS UMR8104, Paris, France, ³Corvus Pharmaceuticals, 863 Mitten Road, Burlingame, Burlingame, CA, ⁴Université Paris Cité, Paris, France
Background/Purpose: T cells abnormal activation plays a pivotal role in the early inflammatory stages of systemic sclerosis (SSc). Compelling evidence supports that SSc has a…

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