Abstracts tagged "Systemic sclerosis"

Abstract Number: 0691 • ACR Convergence 2024
Predictors of Response and Long-term Efficacy and Safety of Nintedanib in Systemic Sclerosis-Interstitial Lung Disease: Data from an Italian Multicentre Study
Corrado Campochiaro¹, Giacomo De Luca², Maria-Grazia Lazzaroni³, Giuseppe Armentaro⁴, Amelia Spinella⁵, Chiara Bellocchi⁶, Barbara Ruaro⁷, Anna Stanziola⁸, Devis Benfaremo⁹, Enrico De Lorenzis¹⁰, Beatrice Moccaldi¹¹, Francesco Bonomi¹², Lorenzo Bianchessi¹³, Florenzo Iannone¹⁴, Fabio Cacciapaglia¹⁵, Lorenzo Beretta¹⁶, Serena Guiducci¹⁷, Veronica Codullo¹⁸, Silvia Laura Bosello¹⁹, Giovanna Cuomo²⁰, Elisabetta Zanatta¹¹, Nicoletta Del Papa⁴, Paolo Airò³, paola Confalonieri⁷, Gianluca Moroncini⁹, Dilia Giuggioli⁵, Lorenzo Dagna²¹ and Marco Matucci-Cerinic²², ¹IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, ²Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan, Milan, Italy, ³Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ⁴Scleroderma Clinic, Dip. Reumatologia, ASST G. Pini-CTO, Milan, Italy, ⁵Scleroderma Unit, University of Modena and Reggio Emilia, Modena, Italy, ⁶Università degli Studi di Milano, IRCCS Ca' Granda Ospedale Maggiore Policlinico di Milano, Milano, Italy, ⁷Unit of Respiratory Medicine, University of Trieste, Trieste, Italy, ⁸Unit of Respiratory Medicine, Federico II University Hospital, Napoli, Italy, ⁹Department of Clinical and Molecular Sciences, Marche Polytechnic University & Department of Internal Medicine, Marche University Hospital, Ancona, Italy, ¹⁰Catholic University of the Sacred Heart, Roma, Rome, Italy, ¹¹Unit of Rheumatology, Padova University, Padova, Italy, ¹²Azienda Ospedaliera Universitaria Careggi, Florence, Florence, Italy, ¹³Unit of Rheumatology, San Matteo Hospital, Pavia, ¹⁴Rheumatology Unit- University of Bari "Aldo Moro", IT, Bari, Italy, ¹⁵Rheumatology Unit � DiMePRe-J, University and AOU Policlinico of Bari, Italy, Bari, Italy, ¹⁶Scleroderma Unit, Fondazione IRCCS Ca' Granda, University of Milan, Milan, Italy, ¹⁷Division of Rheumatology, Scleroderma Unit, Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy, ¹⁸Unit of Rheumatology, San Matteo Hospital, Pavia, Italy, ¹⁹Unit of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, ²⁰Università degli studi della Campania Luigi Vanvitelli, Napoli, Italy, ²¹IRCCS San Raffaele Scientific Institute, Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), Milano, Italy, Milan, Italy, ²²University San Raffaele Milano, Milano, Milan, Italy
Background/Purpose: The SENSCIS trial and the SENSCIS-ON study have shown that nintedanib (NTD) reduces the rate of decline in Interstitial Lung Disease (ILD) in Systemic…
Abstract Number: 0952 • ACR Convergence 2024
Single Cell RNA-seq of Scleroderma-associated Interstitial Lung Disease Lung Explants Reveals an Active, Cytotoxic Natural Killer Cell Population
Cristina Padilla, Eleanor Valenzi, Tracy Tabib, Banafsheh Nazari, Patrizia Fuschiotti and Robert Lafyatis, University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Scleroderma-associated interstitial lung disease (SSc-ILD) is the leading cause of death among SSc patients. Loss of alveolar epithelial cells (AEC) and bronchial cell hyperplasia,…
Abstract Number: 1130 • ACR Convergence 2024
Safety of Bispecific T-cell Engager Therapy in Autoimmune Disease
Melanie Hagen¹, Laura Bucci¹, Sebastian Boeltz², Danae-Mona Noethling², Tobias Rothe², Maria Gabriella Raimondo¹, Carlo Tur¹, Andreas Wirsching¹, Jochen Wacker¹, Aline Bozec¹, Georg Schett³ and Ricardo Grieshaber-Bouyer¹, ¹Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Bayern, Germany, ²Department of Medicine 3 - Rheumatology and Immunology, Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, ³Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: B-cells and plasma cells are key drivers of systemic autoimmune diseases (AID). Recent data demonstrated preliminary safety and efficacy of using the CD3xCD19 bispecific…
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.…

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