Abstracts tagged "Scleroderma, Systemic"

Abstract Number: 1537 • ACR Convergence 2024
Discovery of VENT-03: A Novel Clinical cGAS Inhibitor for the Treatment of SLE and Other Autoimmune Diseases
Kelly A. Pike¹, Nadine Fradet¹, Samuel Gaudreault¹, Alexander M Skeldon², Ramsay E. Beveridge², Patrick Cyr², Nicolas Sgarioto², Philippe Le Gros³, Eleftheria Seliniotakis², Valerie Dumais², Jacklyn Smith², James I.P Stewart², Mehrnaz Gharaee-Kermani⁴, Michelle J Kahlenberg⁴ and Michael A. Crackower², ¹Ventus Therapeutics, Saint-Laurent, QC, Canada, ²Ventus Therapeutics, Saint-Laurent, Canada, ³Ventus Therapeutics, Montreal, QC, Canada, ⁴University of Michigan, Ann Arbor, MI
Background/Purpose: Cyclic GMP-AMP synthase (cGAS) is a nucleic acid sensor that plays a central role in anti-viral responses. Following the detection of intracellular double-stranded DNA,…
Abstract Number: 2449 • ACR Convergence 2024
Acid Reflux Triggers Type I Interferon and Persistent Epithelial-mesenchymal Transition in Esophageal Epithelial Cells. a Novel Microenvironment Contribution to the Pathogenesis of Systemic Sclerosis
Safoura Zahed Mohajerani¹, john ladbury¹, Rebecca Ross² and Francesco Del Galdo¹, ¹University of Leeds, Leeds, United Kingdom, ²Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Gastroesophageal reflux disease (GERD) is a very common manifestation of scleroderma (SSc), affecting as high as 90% of patients, second only to Raynaud’s phenomenon.…
Abstract Number: 1562 • ACR Convergence 2024
Autoantibody Associations with Disease Manifestations in Patients with Early Diffuse Cutaneous Systemic Sclerosis: The Prospective Registry of Early Systemic Sclerosis
Aradhna Agarwal¹, Dahlia Hassan², Suiyuan Huang³, Flavia Castelino⁴, Shervin Assassi⁵, Robyn Domsic⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Monique Hinchcliff¹⁰, Ami Shah¹¹, Victoria Shanmugam¹², Virginia Steen¹³, Dinesh Khanna³ and Elana Bernstein¹⁴, ¹Columbia University, New York, NY, ²Hospital for Special Surgery, New York, NY, ³University of Michigan, Ann Arbor, MI, ⁴Massachusetts General Hospital, Boston, MA, ⁵UTHealth Houston Division of Rheumatology, Houston, TX, ⁶Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Yale School of Medicine, Westport, CT, ¹¹Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ¹²NIH Office of Autoimmune Disease Research in the Office of Research on Women's Health, National Institutes of Health, Bethesda, MD, Bethesda, MD, ¹³Georgetown University School of Medicine, Washington, DC, ¹⁴Columbia University Irving Medical Center, New York, NY
Background/Purpose: Several autoantibodies have been linked to various disease manifestations of systemic sclerosis (SSc). Anti-RNA polymerase 3 (ARA) and anti-topoisomerase-I (ATA) are both associated with…
Abstract Number: 2452 • ACR Convergence 2024
Multimodal Ultrasound in the Assessment of Sarcopenia-related Muscle Involvement in Patients with Systemic Sclerosis: A Multicentric Study
Riccardo Bixio¹, Maria Giovanna Lommano², Seda Colak³, Sonia farah⁴, Francesca Pistillo⁵, Richard Wakefield⁶, Fausto Salaffi⁴, Stefano Di Donato⁷, Ombretta Viapiana⁵, Rossella De Angelis⁸, Maurizio Rossini⁵, Edoardo Cipolletta⁹, Emilio Filippucci¹⁰, Luca Idolazzi⁵, Francesco Del Galdo¹¹ and Andrea Di Matteo¹², ¹University of Verona, Verona, Verona, Italy, ²Università Politecnica delle Marche, Jesi, Italy, ³Gulhane Training and Research Hospital, Ankara, Turkey, ⁴Polytechnic University of Marche, Jesi (AN), Italy, ⁵Rheumatology Unit, University of Verona, Verona, Italy, ⁶NIHR Leeds Biomedical Research Centre, Leeds, UK., Leeds, United Kingdom, ⁷Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ⁸Polytechnic University of Marche, Ancona, Italy, Ancona, Italy, ⁹Polytechnic University of Marche, Ancona, Italy, ¹⁰Rheumatology Unit, Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle Marche, Jesi, Ancona, Italy, Jesi, Italy, ¹¹University of Leeds, Leeds, United Kingdom, ¹²Faculty of Medicine and Health, Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, UK, Leeds, United Kingdom
Background/Purpose: Patients with systemic sclerosis (SSc) are at an increased risk of sarcopenia and muscle involvement. This study aims to describe the ultrasound findings of…
Abstract Number: 0009 • ACR Convergence 2024
Comprehensive Autoantibody Profiling Highlights Clinical Relevance of Autoantibodies to G Protein-coupled Receptors in Systemic Sclerosis: Insights from a B-cell Depletion Clinical Trial
Kazuki Matsuda, Shinichi Sato and Ayumi Yoshizaki, The University of Tokyo, Tokyo, Japan
Background/Purpose: Systemic sclerosis (SSc) affects the skin and internal organs with a poor prognosis. While the exact cause remains unclear, increasing evidence indicates that B…
Abstract Number: 1565 • ACR Convergence 2024
Prevalence and Risk Factors of Lower Extremities Arterial Disease in Systemic Sclerosis: Preliminary Data from a Single Centre Multidisciplinary Study
Maria-Grazia Lazzaroni¹, Paolo Baggi², Liala Moschetti¹, Eleonora Pedretti³, Elda Piovani¹, Claudia Barison¹, Emma Manzoni⁴, Franco Franceschini¹, Stefano Bonardelli² and Paolo Airò¹, ¹Scleroderma Unit, Rheumatology and Clinical Immunology Unit, ERN ReCONNET, ASST Spedali Civili, Brescia, Italy, Brescia, Italy, ²Vascular Surgery Unit, ASST Spedali Civili of Brescia, University of Brescia, Italy, Brescia, Italy, ³Scleroderma Unit, Rheumatology Unit, ERN ReCONNET, ASST Spedali Civili and University of Brescia; Italy, Brescia, Italy, ⁴University of Brescia, Brescia, Italy
Background/Purpose: Microvascular changes represent a key step of pathogenic process in Systemic Sclerosis (SSc). However, SSc has been demonstrated to carry an increased risk of…
Abstract Number: 2455 • ACR Convergence 2024
Improvement Across Multi-organ Domains and Patient Reported Outcomes in Refractory Juvenile-Onset Systemic Sclerosis (jSSc) up to 4 Years After Autologous Stem Cell Transplantation (ASCT)
Jonathan Li¹, Paulina Horvei², Franziksa Rosser³, Kirsten Rose-Felker⁴, Vibha Sood⁵, Adam Olson⁶, Vickie Vandergrift⁷, Nicole Hogue², Lauren Farver⁸, Devin Mcguire⁹, Haley Havrilla⁷, Jessie Alexander¹⁰, Shawna McIntyre², Paul Szabolcs² and Kathryn Torok¹¹, ¹Division of Rheumatology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ²Division of Blood and Marrow Transplantation and Cellular Therapies, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ³Division of Pulmonology Medicine, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA, ⁴Division of Cardiology, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁵Division of Pediatric Gastroenterology, Hepatology and Nutrition, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁶Department of Radiation Oncology, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁷Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁸Department of Physical Therapy, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ⁹Pediatric Behavioral Health, University of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, ¹⁰Pediatric Bone Marrow Transplant and Cellular Therapies, Stanford Children's Hospital, Palo Alto, CA, USA, Pittsburgh, ¹¹Division of Rheumatology, Scleroderma Center, Department of Pediatrics, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, USA, Pittsburgh, PA
Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is a systemic autoimmune disease characterized by vasculopathy and multiorgan fibrosis leading to significant morbidity and early mortality. Autologous stem…
Abstract Number: 0026 • ACR Convergence 2024
Conserved Pro-Fibrotic Fibroblast Signatures in Osteoarthritis Synovium and Systemic Sclerosis Skin
Michael Newton¹, Aanya Mohan², Tareq Hanna³, Dana Orange⁴, John Varga² and Tristan Maerz², ¹University of Michigan, South Lyon, MI, ²University of Michigan, Ann Arbor, MI, ³Wayne State University, Detroit, MI, ⁴The Rockefeller University, New York, NY
Background/Purpose: Synovial fibrosis is a hallmark of osteoarthritis (OA) and a clinical correlate to joint pain. Fibrosis of skin, lungs and other organs is the…
Abstract Number: 1566 • ACR Convergence 2024
Comparative Analysis of Clinical Features and Outcomes in Late Onset vs. Early Onset Scleroderma Renal Crisis – A Multicenter Study
Sumbal Wajid¹, Lee Shapiro², Virginia Steen³, Robyn Domsic⁴, Paul J. Feustel⁵ and Swati Mehta⁶, ¹Albany Medical Center, Albany, NY, ²Division of Rheumatology, Department of Medicine, Albany Medical Center, Albany, NY, ³Georgetown University School of Medicine, Washington, DC, ⁴Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA, ⁵Department of Neuroscience and Experimental Therapeutics, Albany Medical Center, Albany, NY, ⁶Division of Nephrology, Department of Medicine, Albany Medical Center, Albany, NY
Background/Purpose: Scleroderma renal crisis (SRC) is a life-threatening complication of systemic sclerosis (SSc), historically described to occur within the first 5 years of SSc diagnosis.…
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: 0680 • ACR Convergence 2024
Clinical Correlates of Physical and Mental Health in Early Systemic Sclerosis: Data from the Collaborative National Quality and Efficacy (CONQUER) Registry
Ashima Makol¹, Isha Lamba², Dinesh Khanna³, John VanBuren⁴, Angela Child⁴, Jessica Alvey⁴, Shervin Assassi⁵, Elana Bernstein⁶, Flavia Castelino⁷, Lorinda Chung⁸, Luke Evnin⁹, Tracy Frech¹⁰, Faye Hant¹¹, Laura Hummers¹², Kimberly Lakin¹³, Dorota Lebiedz-Odrobina¹⁴, Yiming Luo¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Carrie Richardson¹⁸, Nora Sandorfi¹⁹, Ami Shah²⁰, Ankoor Shah²¹, Brian Skaug⁵, Virginia Steen²², Elizabeth Volkmann²³ and Jessica Gordon²⁴, ¹Mayo Clinic, Rochester, MN, Rochester, MN, ²New York Presbyterian Hospital/Weill Cornell Medical Centre, New York, NY, ³University of Michigan, Ann Arbor, MI, ⁴University of Utah, Salt Lake City, UT, ⁵UTHealth Houston Division of Rheumatology, Houston, TX, ⁶Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁷Massachusetts General Hospital, Boston, MA, ⁸Stanford University, Woodside, CA, ⁹Scleroderma Research Foundation, Brisbane, CA, ¹⁰Vanderbilt University Medical Center, Nashville, TN, ¹¹Medical University of South Carolina, Charleston, SC, ¹²Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹³Hospital for Special Surgery, New York, NY, ¹⁴University of Utah, Cottonwood Heights, UT, ¹⁵Columbia University, New York, NY, ¹⁶University of Minnesota, Minneapolis, MN, ¹⁷Northwestern Memorial Hospital, Chicago, IL, ¹⁸Northwestern University, Chicago, IL, ¹⁹University of Pennsylvania, Philadelphia, PA, ²⁰Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ²¹Duke University, Durham, NC, ²²Georgetown University School of Medicine, Washington, DC, ²³University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles, ²⁴Division of Rheumatology, Weill Cornell Medical College, New York, NY
Background/Purpose: Early years of Systemic sclerosis (SSc) are typically the period of peak disease activity and associated with greater pain, fatigue, sleep disturbance, physical impairment,…
Abstract Number: 1569 • ACR Convergence 2024
Scleroderma Renal Crisis – Different Phenotypes and Patient Trajectories
Katya Dolnikov¹, Kochava Toledano¹, Marius Braun², Doron Markovits¹, Sami Giryes¹, Yonit Tavor¹, Alexandra Balbir-Gurman³ and Yolanda Braun Moscovici¹, ¹Rheumatology Institute, Rambam Health Care Campus, Rappaport Faculty of Medicine, Technion-Israeli Institute of Technology, HAIFA, Israel, ²Liver Insitute, Beilinson Hospital, Petach Tikva, Petach Tikva, Israel, ³Rheumatology Institute, Rambam Health Care Campus and The Rappaport Faculty of |Medicine, Technion, Haifa, Israel
Background/Purpose: Scleroderma renal crisis (SRC) is a life-threatening complication that occurs in a minority of patients, particularly those with diffuse cutaneous systemic sclerosis ( SSc).…
Abstract Number: 2462 • ACR Convergence 2024
A Potent Inhibitor of PAI-1, MDI-2517, Mitigates Disease Severity in Preclinical Models of Systemic Sclerosis
Enming Su¹, Pei-Suen Tsou¹, Mark Warnock², Natalya Subbotina¹, Kris Mann¹, Sirapa Vichaikul¹, Xianying Xing¹, Enze Xing¹, Olesya Plazyo¹, Rachael Wasikowski¹, Lam C. Tsoi³, Mark Weinberg⁴, Cory D. Emal⁵, Dinesh Khanna¹, John Varga¹, Thomas H. Sisson¹, Johann Gudjonsson¹ and Daniel Lawrence², ¹University of Michigan, Ann Arbor, MI, ²Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI, ³Michigan, Dept. of Dermatology, Ann Arbor, MI, ⁴MDI, Ann Arbor, MI, ⁵EMU, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a complex and heterogeneous condition characterized by progressive fibrosis in multiple organs. Currently, there is no known cure for SSc,…
Abstract Number: 0682 • ACR Convergence 2024
Results from Outcomes Selection and Development of a Patient-reported Outcome Measure for a Combined Response Index for Limited Cutaneous SSc: The CRISTAL Project
Alain Lescoat¹, Yen Chen², Susan Murphy³, Nadia Vann², Neda Kortam², Rosemary Gedert², Sue Farrington⁴, Yannick Allanore⁵, David Cella⁶, Lorinda Chung⁷, Philip Clements⁸, Christopher Denton⁹, Francesco Del Galdo¹⁰, Oliver Distler¹¹, Monique Hinchcliff¹², Michael Hughes¹³, Laura Hummers¹⁴, John Pauling¹⁵, Janet Pope¹⁶, Virginia Steen¹⁷, John Varga², Peter Merkel¹⁸, Maya H. Buch¹⁹ and Dinesh Khanna², and all CRISTAL collaborators, ¹CHU Rennes - University Rennes 1, Rennes, France, ²University of Michigan, Ann Arbor, MI, ³University of Michigan, Plymouth, MI, ⁴Scleroderma & Raynaud UK, London, United Kingdom, ⁵Université Paris Cité, Paris, France, ⁶Northwestern University, Chicago, IL, ⁷Stanford University, Woodside, CA, ⁸United States, Los Angeles, CA, ⁹University College London, Northwood, United Kingdom, ¹⁰University of Leeds, Leeds, United Kingdom, ¹¹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland, ¹²Yale School of Medicine, Westport, CT, ¹³Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ¹⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹⁵North Bristol NHS Trust, Bristol, United Kingdom, ¹⁶University of Western Ontario, London, ON, Canada, ¹⁷Georgetown University School of Medicine, Washington, DC, ¹⁸University of Pennsylvania, Philadelphia, PA, ¹⁹Division of Musculoskeletal & Dermatological Sciences, University of Manchester, and NIHR Manchester Biomedical Research Centre, Manchester, United Kingdom
Background/Purpose: Limited cutaneous systemic sclerosis (lcSSc) is the most frequent subset of scleroderma, yet there is a paucity of outcome measures available to assess symptoms…
Abstract Number: 1580 • ACR Convergence 2024
Overt Polyautoimmunity and Its Risk Factors in a Multicenter Cohort of Systemic Sclerosis Patients
Irene Carrión-Barberà¹, Laura Tío², Laura Triginer², María Lee², Alfredo Guillén-del Castillo³, Anna Ribes², Lidia Valencia Muntalà⁴, Jordi Monfort¹, Tarek Carlos Salman Monte:⁵, Carmen PIlar Simeón-Aznar³, Anna Pros¹ and Javier Narvaez-García⁶, ¹Hospital del Mar, Barcelona, Spain, ²Hospital del Mar Research Institute, Barcelona, Spain, ³Hospital Vall d'Hebron, Barcelona, Spain, ⁴Hospital de Bellvitge, Barcelona, Spain, ⁵Hospital del Mar/Parc de Salut Mar-IMIM, Barcelona, Spain, ⁶Hospital Universitario de Bellvitge, Barcelona, Spain
Background/Purpose: Overt PolyA (the clinical coexistence of ≥2 autoimmune diseases (AID) that fulfill classification criteria), is a frequent phenomenon in systemic sclerosis (SSc). However, the…

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