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Abstracts tagged "Systemic sclerosis"

  • Abstract Number: 2656 • ACR Convergence 2025

    Long-term effect of selexipag in systemic sclerosis-associated digital ulcers: a case control, multicentre, observational study

    Claudia Iannone1, Marco Di Battista2, Maria Rosa Pellico3, ilaria Magi4, Antonina Minniti5, Giuseppe Armentaro4, Silvia Cavalli4, Manuel Sette4, Laura Giudice4, Cristina Bochicchio4, Alessandra Della Rossa6, Antonio Gaetano Tavoni7, Fabio Cacciapaglia8, Stefano Stano9, Martina Orlandi10, Dilia Giuggioli11, Marta Mosca12, Roberto Caporali13 and Nicoletta Del pAPA3, 1University of Milan, Gaetano Pini CTO, Milano, Milan, Italy, 2Rheumatology Unit, University of Pisa, Pisa, Italy, 3ASST Pini-CTO, Clinical Rheumatology Unit, Milano, Italy, 4ASST Pini-CTO, Clinical Rheumatology Unit, Milan, Italy, 5ASST Gaetano Pini, milano, Milan, Italy, 6Rheumatology Unit, University of Pisa, Milan, Italy, 7Clinical Immunology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Milan, Italy, 8Rheumatology Unit DiMePRe-J, University and AOU Policlinico of Bari & Department of Medicine LUM "G. De Gegnnaro" University, Casamassima (Bari) - Italy, Bari, Italy, 96. Rheumatology Unit, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari, Bari, Italy, 10Rheumatology Unit, Department of Medical and Surgical Sciences for Children and Adults, University Hospital of Modena and Reggio Emilia School of Medicine, Italy., modena, Italy, 11Scleroderma Unit, Rheumatology Unit, University Hospital of Modena and Reggio Emilia, Modena, Italy, Modena, Italy, 12University of Pisa, Pisa, Pisa, Italy, 13University of Milan and ASST Gaetano Pini-CTO, Milano, Italy

    Background/Purpose: Digital ulcers (DUs) affect approximately 50% of SSc patients, causing significant pain and disability. Current management involves both systemic and local therapies. However, the…
  • Abstract Number: 2484 • ACR Convergence 2025

    Phenotype of systemic sclerosis patients with positive anti–NOR-90 antibodies

    Javier Narváez1, Santos Castañeda2, Montserrat Roig Kim3, Martí Aguilar-Coll4, Laia De Daniel Bisbe3, Marina Dueñas5, Cristina Valero6, Francisco Morandeira7, Miguel A. González-Gay8 and Joan Miquel Nolla3, 1Hospital Universitario de Bellvitge, Barcelona, Spain, 2Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Madrid, Spain, 3Hospital Universitari de Bellvitge, L'Hospitalet de Llobregat, Spain, 4Department of Rheumatology. Hospital Universitario de Bellvitge, Barcelona, Spain, 5Department of Rheumatology.Hospital Universitario de la Princesa, Madrid, Spain, 6Hospital de la Princesa, Madrid, Spain, 7Department of Immunology. Hospital Universitario de Bellvitge, Barcelona, Spain, 8Department of Rheumatology and Joint and Bone Research Unit, Hospital Universitario Fundación Jiménez Díaz, and Instituto de Investigación Sanitaria-Fundación Jiménez Díaz, Madrid, Spain, and Medicine and Psychiatry Department, University of Cantabria, Santander, Spain

    Background/Purpose: To analyze the clinical associations of anti-Nucleolar Organizer Region 90 antibodies (NOR90) antibodies in patients with systemic sclerosis (SSc)Methods: Cross-sectional study of NOR90-positive SSc…
  • Abstract Number: 1873 • ACR Convergence 2025

    TGF-β-Driven Mitochondrial Stress Activates cGAS-STING Signalling via Impaired Mitophagy in Systemic Sclerosis Endothelial Cells

    Stefano Di Donato1, Ceclie Bordes2, Claude Lalou2, agathe Depaire2, John Tchen2, Charlene Lhuissier2, damien Brisou2, Vanja Sisirak2, Johan Garaude2, Christopher Wasson3, Rebecca Ross4, Francesco Del Galdo3 and Marie-Elise Truchetet5, 1University of Leeds, Canosa Sannita, Chieti, Italy, 2University of Bordeaux, Bordeaux, France, 3University of Leeds, Leeds, United Kingdom, 4Medicine and Health, University of Leeds, Leeds, United Kingdom, 5Bordeaux University Hospital, Bordeaux, France

    Background/Purpose: Emerging evidence implicates mitochondrial dysfunction as a contributor to tissue fibrosis in systemic sclerosis (SSc) (1). Endothelial cells, which are a key player in…
  • Abstract Number: 1583 • ACR Convergence 2025

    Epidemiology, Outcome and Expenditures of Hospitalized Patients with Systemic Sclerosis: National Inpatient Sample 2021

    Patompong Ungprasert1 and Paul Kroner2, 1Cleveland Clinic, Cleveland, OH, Cleveland Heights, OH, 2Riverside Health System, Newport News, VA

    Background/Purpose: The inpatient epidemiology, morbidity, mortality, and healthcare expenditures of patients with systemic sclerosis (SSc) are not well-characterized. This study aims to utilize a nationwide…
  • Abstract Number: 1555 • ACR Convergence 2025

    Prevalence of Organ Involvement and Baseline Predictors of Disease Progression in Patients with Limited Cutaneous Systemic Sclerosis: Insights from the CONQUER Database

    Alain Lescoat1, Virginia Steen2, Monica Harding3, John VanBuren3, Brian Skaug4, Shervin Assassi5, Maureen Mayes6, Zsuzsanna McMahan7, Elana Bernstein8, Flavia Castelino9, Lorinda Chung10, Luke Evnin11, Tracy Frech12, Jessica Gordon13, Faye Hant14, Laura Hummers15, Kimberly Lakin13, Dorota Lebiedz-Odrobina3, Yiming Luo16, Ashima Makol17, Jerry Molitor18, Duncan Moore19, Carrie Richardson19, Nora Sandorfi20, Ami Shah15, Ankoor Shah21, Elizabeth Volkmann22, Carleigh Zahn23 and Dinesh Khanna24, 1CHU Rennes - University Rennes, Rennes, France, 2Georgetown University School of Medicine, Washington, DC, 3University of Utah, Salt Lake City, UT, 4UTHealth Houston Division of Rheumatology, Houston, TX, 5Division of Rheumatology, UTHealth Houston, Houston, Texas, USA, Houston, TX, 6UT Health Houston Division of Rheumatology, Houston, TX, 7UT Health Houston, Houston, TX, 8Columbia University Irving Medical Center, New York, NY, 9Massachusetts General Hospital, Boston, MA, 10Stanford University, Stanford, CA, 11Scleroderma Research Foundation, San Francisco, CA, 12Vanderbilt University Medical Center, Nashville, TN, 13Hospital for Special Surgery, New York, NY, 14Medical University of South Carolina, Charleston, SC, 15Johns Hopkins Rheumatology, Baltimore, MD, 16Columbia University Irving Medical Center, New York, 17Mayo Clinic, Rochester, MN, 18University of Minnesota, Minneapolis, MN, 19Northwestern University, Chicago, 20University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, 21Duke University, Durham, NC, 22Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA, Los Angeles, CA, 23University of Michigan, Ann Arbor, 24University of Michigan, Ann Arbor, MI

    Background/Purpose: Systemic sclerosis (SSc) is characterized by a wide range of clinical manifestations and available interventions still lack overall efficacy. Limited cutaneous SSc (lcSSc) and…
  • Abstract Number: 0971 • ACR Convergence 2025

    Unraveling the role of the hippo pathway in systemic sclerosis: A focus on TEADs and VGLL3

    Neha Khanna1, Alyssa Rosek1, Alexander Cai1, Olesya Plazyo2, Johann Gudjonsson2, Dinesh Khanna2 and Pei-Suen Tsou2, 1Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, 2University of Michigan, Ann Arbor, MI

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disorder characterized by excessive collagen production, leading to the thickening and hardening of skin and internal organs.…
  • Abstract Number: 0704 • ACR Convergence 2025

    Human Pharmacokinetics of Inhaled Liposomal Iloprost Support a Dosing Regimen for Treating Systemic Sclerosis- Related Digital Ulcer

    Pei Kan, Ko-Chieh Chen and Shin-Shin Chuang, Pharmosa Biopharm Inc, Taipei, Taiwan (Republic of China)

    Background/Purpose: Iloprost is recommended and widely used in Europe for treating systemic sclerosis (SSc)-related Raynaud Phenomena (RP) and Digital Ulcer (DU). EULAR recommendation suggests continuous…
  • Abstract Number: 0675 • ACR Convergence 2025

    Neutrophil-to-Lymphocyte Ratio as a biomarker for disease onset and a predictor for mortality in Systemic Sclerosis – real-world data from a large healthcare provider in Israel.

    Shiri Keret1, Shlomit Yaari2, gleb Slobodin3 and doron Rimar3, 1Bnai Zion Medical Center, Atlit, Israel, 2Maccabi Healthcare Services, HaMered 27, Tel Aviv, 68125, Israel., Tel Aviv, Israel, 3Bnai Zion Medical Center Faculty of Medicine, Technion Israel institute of technology, Haifa, Israel

    Background/Purpose: Elevated neutrophil-to-lymphocyte ratio (NLR) in systemic sclerosis (SSc) has been demonstrated to predict worsening skin and lung involvement, and a cutoff of 2.95 was…
  • Abstract Number: 0810 • ACR Convergence 2025

    Spatial Transcriptomic-based Phenotyping of the Fibroblast Niches in Systemic Sclerosis-associated Primary Heart Involvement

    Alexandru Micu1, Alexandru-Emil Matei2, Yi-Nan Li3, Ann-Christin Pecher4, Tim Filla5, Jörg Henes6, Markus Eckstein7, Karin Klingel8, Jörg Distler9 and Andrea-Hermina Györfi10, 1Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University, Düsseldorf, Germany, 2Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Fraunhofer Institute for Translational Medicine and Pharmacology ITMP, and Fraunhofer Cluster of Excellence for Immune Mediated Diseases CIMD, Frankfurt am Main, Germany, Düsseldorf, Germany, 3University Hospital of Düsseldorf, Düsseldorf, Germany, 4Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tübingen, Germany, 5Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University. Hiller Research Center, University Hospital Düsseldorf, Medical Faculty of Heinrich-Heine University., Düsseldorf, Germany, 6Department of Internal Medicine II, Hematology, Oncology, Clinical Immunology, and Rheumatology, University Hospital Tübingen, Tuebingen, Germany, 7Institute of Pathology and Comprehensive Cancer Center EMN, Friedrich-Alexander- Universität (FAU) Erlangen-Nürnberg and Uniklinikum Erlangen, Erlangen, Germany, 8Cardiopathology, Institute for Pathology, Eberhard-Karls-Universität Tübingen, Tübingen, Germany, 9University Hospital Duesseldorf and HHU, Duesseldorf, Germany, 10Department of Rheumatology, University Hospital Düsseldorf, Medical Faculty of Heinrich Heine University., Düsseldorf, Germany

    Background/Purpose: Systemic sclerosis (SSc)-associated primary heart involvement (SSc-pHI) is one of the leading causes of mortality in SSc, yet its underlying cellular and molecular pathomechanisms…
  • Abstract Number: 2652 • ACR Convergence 2025

    Anti-mitochondrial antibodies in systemic sclerosis target enteric neurons and are associated with GI dysmotility

    Zsuzsanna McMahan1, Srinivas Puttapaka2, Livia Casciola-Rosen3, Timothy Kaniecki3, Laura Gutierrez3, Su Hong MIng2, Philippa Seika2 and Subhash Kulkarni4, 1UT Health Houston, Houston, TX, 2BIDMC/Harvard, Boston, MA, 3Johns Hopkins University, Baltimore, MD, 4BIDMC, Boston, MA

    Background/Purpose: Most patients with systemic sclerosis (SSc) experience gastrointestinal (GI) dysmotility. The enteric nervous system (ENS) regulates GI motility, and its dysfunction causes dysmotility. A…
  • Abstract Number: 2483 • ACR Convergence 2025

    Is There a Seasonal Variation in Raynaud’s Phenomenon Severity in Patients with Systemic Sclerosis?

    Gulsen Ozen1, Lan Yu2, Maureen Lauffoon2, John Pauling3 and Robyn Domsic2, 1University of Iowa Carver College of Medicine, Coralville, IA, 2University of Pittsburgh, Pittsburgh, PA, 3North Bristol NHS Trust, Bristol, United Kingdom

    Background/Purpose: Raynaud’s phenomenon (RP) is the most common manifestation of systemic sclerosis (SSc) leading to substantial pain and impaired hand function. Establishing treatment efficacy for…
  • Abstract Number: 1871 • ACR Convergence 2025

    Examining the impact of ETV2 on altered endothelial phenotype in systemic sclerosis

    Elio Carmona1, Alyssa Rosek2, Neha Khanna2, Dinesh Khanna3, Amr Sawalha4 and Pei-Suen Tsou3, 1Division of Pediatric Rheumatology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA, Pittsburgh, 2Division of Rheumatology, Department of Internal Medicine, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, 3University of Michigan, Ann Arbor, MI, 4University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Our prior research demonstrated that epigenetic modifications are central to the pathogenesis of scleroderma (SSc)1. Using ATAC-seq, we found that chromatin accessibility was significantly…
  • Abstract Number: 1582 • ACR Convergence 2025

    Risk factors for incident digital ischemic complications in systemic sclerosis in the Collaborative National Quality and Efficacy Registry (CONQUER)

    Marissa Savoie1, Monica Harding2, John VanBuren2, Shervin Assassi3, Elana Bernstein4, Lorinda Chung5, Luke Evnin6, Tracy Frech7, Jessica Gordon1, Faye Hant8, Laura Hummers9, Dinesh Khanna10, Kimberly Lakin1, Dorota Lebiedz-Odrobina2, Yiming Luo4, Ashima Makol11, Maureen Mayes12, Zsuzsanna McMahan13, Jerry Molitor14, Duncan Moore15, Carrie Richardson16, Nora Sandorfi17, Ami Shah9, Ankoor Shah18, Brian Skaug19, Virginia Steen20, Elizabeth Volkmann21, Carleigh Zahn10 and Flavia Castelino22, 1Hospital for Special Surgery, New York, NY, 2University of Utah, Salt Lake City, UT, 3Division of Rheumatology, UTHealth Houston, Houston, Texas, USA, Houston, TX, 4Columbia University, New York, NY, 5Stanford University, Stanford, CA, 6Scleroderma Research Foundation, Brisbane, CA, 7Vanderbilt University Medical Center, Nashville, TN, 8Medical University of South Carolina, Charleston, SC, 9Johns Hopkins Rheumatology, Baltimore, MD, 10University of Michigan, Ann Arbor, MI, 11Mayo Clinic, Rochester, MN, 12UT Health Houston Division of Rheumatology, Houston, TX, 13UT Health Houston, Houston, TX, 14University of Minnesota, Minneapolis, MN, 15Northwestern Memorial Hospital, Chicago, IL, 16Northwestern University, Chicago, IL, 17University of Pennsylvania, Philadelphia, PA, 18Duke University, Durham, NC, 19UTHealth Houston Division of Rheumatology, Houston, TX, 20Georgetown University School of Medicine, Washington, DC, 21Division of Rheumatology, Department of Medicine, University of California, David Geffen School of Medicine, Los Angeles, CA, USA, Los Angeles, CA, 22Massachusetts General Hospital, Boston, MA

    Background/Purpose: There have not been large US-based studies of digital pitting scars (DPS) and digital ischemic ulcers (DIU) in systemic sclerosis. Utilizing the Collaborative National…
  • Abstract Number: 1553 • ACR Convergence 2025

    Circulating Monocyte Level Is Associated With Pulmonary Vascular Disease and Right Ventricular Dysfunction in Systemic Sclerosis

    Ai Yaku1, Bong Joon Kim2, Hidenori Yaku1, Carrie Richardson2, Michael Cuttica2, Ruben Mylvaganam2, Vera Rigolin2, Matthew Feinstein2 and Sanjiv Shah1, 1Northwestern University, Chicago, IL, 2Northwestern University, Chicago

    Background/Purpose: Pulmonary arterial hypertension associated with systemic sclerosis (SSc-PAH) is a life-threatening condition with obliterative pulmonary vascular remodeling, right ventricular (RV) dysfunction, and poor prognosis.…
  • Abstract Number: 0970 • ACR Convergence 2025

    Genomic instability in systemic sclerosis is promoted by metabolic remodelling via a FOXO1-dependent axis

    Lamia Khan1, Junqin Wang2, Aishwarya Iyer2, Desiree Redmond2, Dylan Hennessey2, Sandra O'Keefe2, Jan Storek3, Charmaine van Eeden2, Robert Gniadecki2 and Mohammed Osman1, 1University of Alberta, Edmonton, AB, Canada, 2Department of Medicine, University of Alberta, Edmonton, AB, Canada, 3University of Calgary, Calgary, AB, Canada

    Background/Purpose: Systemic sclerosis (SSc) is a life-threatening autoimmune disease with limited treatment options, including autologous hematopoietic stem cell transplantation (AHSCT). We recently showed that dermal…
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