Abstracts tagged "Systemic sclerosis"

Abstract Number: 0690 • ACR Convergence 2024
Can Immunosuppression or Vasodilation Prevent the Onset of Interstitial Lung Disease in Systemic Sclerosis? An Association Analysis from the EUSTAR Database
Liubov Petelytska¹, Lorenzo Tofani², Elise Siegert³, Elisabetta Zanatta⁴, Gianluca Moroncini⁵, Christina Bergmann⁶, vanessa smith⁷, Gabriela Riemekasten⁸, Kamal Solanki⁹, Fabio Cacciapaglia¹⁰, Britta Maurer¹¹, José da Silva¹², Marija Geroldinger-Simić¹³, Ana Rodrigues¹⁴, Madelon Vonk¹⁵, Jeska de Vries-Bouwstra¹⁶, Anna-Maria Hoffmann-Vold¹⁷, Oliver Distler¹⁸ and Cosimo Bruni¹⁹, and on behalf of the EUSTAR collaborators, ¹Bogomolets National Medical University, Kyiv, Kyyiv, Ukraine, ²University of Florence, Florence, Italy, ³Charité University Hospital, Berlin, Germany, ⁴Unit of Rheumatology, Padova University, Padova, Italy, ⁵Marche Polytechnic University, Ancona, Italy, ⁶Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁷Ghent University Hospital, Gent, Belgium, ⁸University of Lübeck, Department of Rheumatology and Clinical Immunology, Lübeck, Germany, ⁹Waikato Hospital, Hamilton, New Zealand, ¹⁰Rheumatology Unit DiMePRe-J, University and AOU Policlinico of Bari, Italy, Bari, Italy, ¹¹University Hospital Bern, University Bern, Bern, Switzerland, ¹²University of Coimbra, Coimbra, Portugal, ¹³Johannes Kepler University, Linz, Austria, ¹⁴Hospital Curry Cabral-Centro Hospitalar Universitário Lisboa Central, Lisbon, Portugal, ¹⁵Radboud University Nijmegen Medical Centre, Nijmegen, Netherlands, ¹⁶Leiden University Medical Center, Leiden, Netherlands, ¹⁷Oslo University Hospital, Oslo, Norway, ¹⁸Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, ¹⁹University of Zurich, Zurich, Switzerland
Background/Purpose: Inflammation and vasculopathy may play a role in the development of interstitial lung disease (ILD) in systemic sclerosis (SSc). Pre-clinical evidence showed possible preventive…
Abstract Number: 0951 • ACR Convergence 2024
A Feedback Loop Involving mtDNA, Platelets and Neutrophil Extracellular Traps Amplifies Inflammation in Systemic Sclerosis
Stavros Giaglis¹, Diego Kyburz² and Ulrich Walker³, ¹Experimental Rheumatology Laboratory, University of Basel, Basel, Switzerland, ²University Hospital Basel, Basel, Switzerland, ³Basel University Hospital, Basel, Switzerland
Background/Purpose: Mitochondria are essential eucaryotic cell organelles with several bacterial features, such as a double-stranded circular genome with hypomethylated CpG areas (1). A fundamental role of…
Abstract Number: 1070 • ACR Convergence 2024
Evaluating & Improving Adherence to Pulmonary Hypertension Screening in Systemic Sclerosis Patients
John Hickernell¹ and Zineb Aouhab², ¹Loyola Univeristy Medical Center, Chicago, IL, ²Loyola University Medical Center, Oak Brook, IL
Background/Purpose: Annual screening for pulmonary hypertension (PH) in patients with Systemic Sclerosis (SSc) has been shown to decrease mortality. The American College of Radiology, European…
Abstract Number: 1577 • ACR Convergence 2024
Elevated Neutrophil Extracellular Traps in Systemic Sclerosis-associated Vasculopathy and Suppression by a Synthetic Prostacyclin Analog
Neda Kortam¹, Wenying Liang¹, Claire Shiple¹, Suiyuan Huang¹, Rosemary Gedert¹, James St. Clair¹, Cyrus Sarosh², Caroline Foster¹, Eliza Pei-Suen Tsou¹, John Varga¹, Jason Knight¹, Dinesh Khanna¹ and Ramadan Ali¹, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan, Temperance, MI
Background/Purpose: Neutrophils and neutrophil extracellular traps (NETs) contribute to the vascular complications of multiple diseases, but their role in systemic sclerosis (SSc) is understudied. We…
Abstract Number: 1817 • ACR Convergence 2024
Activated Macrophages Mediate Loss of Dermal White Adipose Tissue in Fibrotic Skin
Chanhyuk Park¹, Helen Jarnagin², Asmaa Mohamed³, Yina Huang³, Michael Whitfield⁴ and Patricia Pioli¹, ¹Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth College, Lebanon, NH, ³Dartmouth, Lebanon, ⁴Geisel School of Medicine at Dartmouth, Hanover, NH
Background/Purpose: Autoimmune systemic sclerosis (SSc) is characterized by inflammation, vasculopathy, and dermal and internal organ fibrosis. A widely-reported but poorly understood aspect of SSc skin…
Abstract Number: 2183 • ACR Convergence 2024
Accuracy of AI Tools for Diagnosis of Connective Tissue Disease
Indira Sriram¹, Gabriel Tarshish² and Megan Curran², ¹University of Colorado, Aurora, CO, ²Children's Hospital Colorado, Aurora, CO
Background/Purpose: In this work, we study the ability of generative artificial intelligence (AI) based tools to diagnose pediatric rheumatological diseases. Specifically, we seek to answer…
Abstract Number: 2458 • ACR Convergence 2024
Risk Factors for Progression of Interstitial Lung Disease in Patients with Early Diffuse Cutaneous Systemic Sclerosis
Julie Urgiles¹, Suiyuan Huang², Shervin Assassi³, Flavia Castelino⁴, Robyn Domsic⁵, Tracy Frech⁶, Jessica Gordon⁷, Faye Hant⁸, Monique Hinchcliff⁹, Ami Shah¹⁰, Victoria Shanmugam¹¹, Virginia Steen¹², Dinesh Khanna² and Elana Bernstein¹³, ¹Columbia University Irving Medical Center, New York, NY, ²University of Michigan, Ann Arbor, MI, ³UTHealth Houston Division of Rheumatology, Houston, TX, ⁴Massachusetts General Hospital, Boston, MA, ⁵Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁶Vanderbilt University Medical Center, Nashville, TN, ⁷Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁸Medical University of South Carolina, Charleston, SC, ⁹Yale School of Medicine, Westport, CT, ¹⁰Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ¹¹Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, ¹²Georgetown University School of Medicine, Washington, DC, ¹³Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY
Background/Purpose: Interstitial lung disease (ILD) is one of the main pulmonary complications of systemic sclerosis (SSc), with a prevalence of 50-60%. SSc-ILD progression can occur…
Abstract Number: 2686 • ACR Convergence 2024
Dose Escalation Safety Study of Brentuximab Vedotin for Diffuse Cutaneous Systemic Sclerosis:Clinical Results and Mechanistic Analysis of Skin and Peripheral Blood Mononuclear Cells
David Fox¹, Laura Cooney², Lia Stelzig³, Robert Lafyatis⁴, Johann Gudjonsson⁵, Lam Tsoi⁶, Maureen Mayes⁷, Ankoor Shah⁸, Suzanne Kafaja⁹, William Barry³, Ellen Goldmuntz¹⁰, Dawn Smilek¹¹ and Dinesh Khanna⁶, ¹University of Michigan, Dexter, MI, ²University of Michigan and Immune Tolerance Network, Ann Arbor, MI, ³Rho, Durham, NC, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh, Pittsburgh, PA, ⁵Department of Dermatology, University of Michigan, Ann Arbor, MI, ⁶University of Michigan, Ann Arbor, MI, ⁷UTHealth Houston Division of Rheumatology, Houston, TX, ⁸Duke University, Durham, NC, ⁹Division of Rheumatology, University of California, Los Angeles, Los Angeles, CA, ¹⁰Division of Allergy, Immunology, and Transplantation, National Institute of Allergy and Infectious Diseases, Rockville, MD, ¹¹Immune Tolerance Network, UCSF, San Francisco, CA
Background/Purpose: Brentuximab vedotin (ADCETRIS®) is a drug antibody conjugate targeting CD30 approved for treatment of Hodgkin’s lymphoma (HL) and other hematologic malignancies. Association of CD30…
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…

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