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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 1578 • ACR Convergence 2024

    Association of Systemic Sclerosis and Other Systemic Autoimmune Disease Diagnosis with Increasing Anti-RNA Polymerase III Antibodies

    Warren Balaja1, Mashood Badshah2 and Megan Krause3, 1University of Kansas Medical Center, Kansas City, KS, 2The University of Kansas Medical Center, Kansas City, KS, 3University of Kansas, Kansas City, KS

    Background/Purpose: Anti-RNA polymerase III antibody (ARA) is a disease-specific antibody important for the identification and stratification of varying phenotypes of systemic sclerosis (SSc). Previous studies…
  • 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: 2197 • ACR Convergence 2024

    Outcome Measures for the Assessment of Cutaneous and Musculoskeletal Manifestations of Juvenile Systemic Sclerosis: An International Collaborative Scoping Review and Survey of Current Practice

    Amanda Robinson1, Mustafa Çakan2, Simone Appenzeller3, Susan Shenoi4, Meiping Lu5, Betul Sozeri6, Rongjun Zheng5, Priya Bhave7, Natalia Vasquez Canizares8 and Suzanne Li9, and International Juvenile Systemic Sclerosis Outcomes Group (IJOG), 1Division of Pediatric Rheumatology, University of Utah, Salt Lake City, UT, 2Clinic of Pediatric Rheumatology, Zeynep Kamil Women and Children's Diseases Training and Research Hospital, İstanbul, Turkey, 3Unicamp, Campinas, SP, Brazil, 4Seattle Children's Hospital and Research Center, Mercer Island, WA, WA, 5Department of Rheumatology Immunology and Allergy, Children's Hospital of Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China (People's Republic), 6University of Health Sciences, Istanbul, Turkey, 7Hackensack Meridian School of Medicine, Hackensack, NJ, 8Children's Hospital at Montefiore; Albert Einstein College of Medicine;, New York, NY, 9Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is a rare fibrosing autoimmune disease associated with significant morbidity and mortality. Data on treatment strategies is limited, primarily based…
  • Abstract Number: 2459 • ACR Convergence 2024

    Use of Nailfold Capillaroscopy as a Predictor of Cardiovascular Events and Mortality in Systemic Sclerosis

    Carlos Valera-Ribera1, Javier Narvaez-García2, Joaquín Lacasa-Molina3, Laura Perea-Galera4, Alida Taberner-Cortés3, Paula Andrea Mencio-Viana3, Patricia Leon-Torralba3, Adrián Mayo-Juanatey3, Pablo Andújar-Brazal3, Ignacio Vázquez Gómez5, Desamparados Ybáñez-García3, Àngels Martínez-Ferrer6, Elia Valls-Pascual7, Montserrat Robustillo Villarino8 and JUAN JOSE ALEGRE SANCHO3, 1Hospital Doctor Peset, Valéncia, Comunidad Valenciana, Spain, 2Hospital Universitario de Bellvitge, Barcelona, Spain, 3Hospital Universitario Doctor Peset, Valencia, Spain, 4Hospital Universitario Doctor Peset, Valencia, 5H. U. Dr. Peset, Valencia, 6Hospital Universitario Dr Peset Valéncia, Valéncia, Spain, 7Hospital General de Valencia, Valencia, Comunidad Valenciana, Spain, 8Hospital Universitario La Plana, Villarreal, Spain

    Background/Purpose: Systemic sclerosis (SSc) is a disease with high morbidity and mortality. Nailfold capillaroscopy (NFC) is a widely used method for its diagnosis. Our objective…
  • Abstract Number: PP07 • ACR Convergence 2024

    My Journey with Diffuse Scleroderma and Interstitial Lung Disease: A Pharmacist’s Perspective

    Joseph Washington, Cencora, Washington, DC

    Background/Purpose: Since my diagnosis with diffuse scleroderma and ILD, I have balanced the roles of patient and young pharmacist. A duality that has surely impacted…
  • Abstract Number: 0697 • ACR Convergence 2024

    Association of ANCA-Associated Vasculitis and Development of SSc and SSc-Associated Antibodies: 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: Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a spectrum of autoimmune disorders characterized by necrotizing small-to-medium vessel inflammation and circulating ANCAs. Represented by granulomatosis…
  • 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,…
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