ACR Meeting Abstracts

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Abstracts tagged "Scleroderma, Systemic"

  • Abstract Number: L01 • ACR Convergence 2024

    Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases

    Matthew Martin1, Erik Wilker1, Diana Gikunju1, Usha Narayanan1, Unnati Pandya1, Vijetha Prakash1, Ashley Edwards1, Sameer Kawatkar1, Tenghui Chen1, Ragunath Chandran1, Sai Sunder1, Sumathi Biradar1, Joerg Distler2, Alexandra Joseph1, Stephanos Ioannidis1 and Bhavatarini Vangamudi1, 1Exo Therapeutics, Cambridge, MA, 2Exo Therapeutics, Düsseldorf, Germany

    Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…
  • 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…
  • Abstract Number: 2463 • ACR Convergence 2024

    Interstitial Lung Disease in Very Early Systemic Sclerosis – a Clinical and Radiological Characterization

    Sinziana Muraru1, Christian Blüthgen2, Cosimo Bruni1, Carina Mihai1, Anna-Maria Hoffmann-Vold3, Muriel Elhai4, Mike Becker5, Thomas Frauenfelder6, Oliver Distler7 and Rucsandra Dobrota1, 1University Hospital Zurich, University of Zurich, Zurich, Switzerland, 2Institute for Diagnostic and Interventional Radiology, University Hospital Zurich, Zurich, Switzerland, 3Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 4University Hospital zurich, Zürich, Switzerland, 5Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 6University Hospital Zurich, Zurich, Zurich, Switzerland, 7Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland

    Background/Purpose: Interstitial lung disease (ILD) occurs in about 50% of patients with systemic sclerosis (SSc). The aims of this study were to assess the prevalence…
  • Abstract Number: 0683 • ACR Convergence 2024

    Prognostic Value of Systemic Sclerosis-associated Primary Heart Involvement

    Caya Gharibian1, Vanessa Lupi1, Alexander Gotschy1, Mike Becker2, Rucsandra Dobrota1, Muriel Elhai3, Sinziana Muraru1, Suzana Jordan1, Anna-Maria Hoffmann-Vold4, Oliver Distler5, Robert Manka1, Cosimo Bruni1 and Carina Mihai1, 1University Hospital Zurich, University of Zurich, Zurich, Switzerland, 2Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 3University Hospital zurich, Zürich, Switzerland, 4Oslo University Hospital, Oslo, Norway, 5Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland

    Background/Purpose: A definition of primary heart involvement (pHI) in systemic sclerosis (SSc) was recently developed (Bruni C et al. J Scleroderma Relat Disord. 2022;7:24-32). Cardiac…
  • Abstract Number: 1584 • ACR Convergence 2024

    Efficacy of Tofacitinib in the Treatment of Early Diffuse Cutaneous Systemic Sclerosis: A Clinical Study in Bangladesh

    Md Zahid Amin1, Nabil Amin khan2, Prof. Md. Abu Shahin2, Dr. Md. Ariful Islam2, Prof. Md. Nazrul Islam2, Prof. Syed Atiqul Haq3, Prof. Minhaj Rahim Choudhury3 and Prof. Mohammad Mostafa Zaman4, 1Central Police Hospital, Dhaka, Dhaka, Bangladesh, 2BSMMU, Dhaka, Dhaka, Bangladesh, 3Department of Rheumatology, BSMMU, Dhaka, Dhaka, Bangladesh, 4Department of Public Health and Informatics, BSMMU, Dhaka, Dhaka, Bangladesh

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis, vascular abnormalities, and immune system dysregulation. Current treatment options are limited, and there…
  • Abstract Number: 2472 • ACR Convergence 2024

    Are Anti-centromere Antibodies (aCENP) Predictive of Systemic Sclerosis Development in Patients Without Raynaud´s Phenomenon?

    Andrea Alvear-Torres1, María José Martínez-Becerra2, María Carmen Vegas-Sánchez3, Sheila Recuero-Díaz1, María Pérez-ferro4 and Olga Sánchez-Pernaute5, 1Department of Rheumatology and Joint and Bone Research Unit. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, 2Department of Immunology. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, 3Hospital Universitario Fundación Jiménez Díaz and IIS-FJD, Madrid, Spain, 4Department of Rheumatology. Hospital Universitario Rey Juan Carlos, Madrid, Spain, 5Department of Rheumatology and Joint and Bone Research Unit. Hospital Universitario Fundación Jiménez Díaz and IIS-FJD. Autonomous University of Madrid, Madrid, Spain

    Background/Purpose: Anti-centromere (aCENP) antibodies are considered highly specific of systemic sclerosis (SSc), typically defining a limited cutaneous phenotype, usually associated to Raynaud´s phenomenon (RP). It…
  • Abstract Number: 0684 • ACR Convergence 2024

    The Diagnostic Accuracy of Ultrasound for Low Muscle Mass in Women with Systemic Sclerosis

    Lucas Dória1, Vanessa Hax2, André Mallmann2, Rafaela Santo3, Leonardo Santos2, Stephanie Pilotti2, Daniel Moraes2, Isabella Boening2, Pedro Glanze2, ODIRLEI MONTICIELO1, Ricardo Xavier4 and Rafael Chakr5, 1Hospital de Clinicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil, 2Hospital de Clínicas de Porto Alegre, Porto Alegre, Rio Grande do Sul, Brazil, 3Health Research and Innovation Science Centre, Klaipeda University, Klaipeda, Lithuania, Porto Alegre, Rio Grande do Sul, Brazil, 4Universidade Federal do Rio Grande do Sul, Porto Alegre/RS, Rio Grande do Sul, Brazil, 5Universidade Federal do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil

    Background/Purpose: Patients with systemic sclerosis (SSc) often experience body composition changes, including low muscle mass (myopenia), associated with physical disability, hospitalization and death. Our purpose…
  • Abstract Number: 1585 • ACR Convergence 2024

    Characterization of the Fecal Metabolome in Early Systemic Sclerosis

    Arissa Young1, Kristofer Andreasson2, Jen Labus3, Nedas Matulianos4, Blake Wilde1, Jonathan Jacobs3, Heather Christofk4 and Elizabeth Volkmann5, 1UCLA, Los Angeles, CA, 2Lund University, Lund, Sweden, 3UCLA, Los Angeles, 4University of California Los Angeles, Los Angeles, CA, 5University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles

    Background/Purpose: Previous studies have shown that alterations in gut microbiota are present early in systemic sclerosis (SSc) (Andréasson et al. 2022) and are associated with…
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