ACR Meeting Abstracts

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Abstracts tagged "Scleroderma"

  • Abstract Number: 029 • 2023 Pediatric Rheumatology Symposium

    Cardiac Magnetic Resonance Imaging in Children with Systemic Lupus Erythematosus and Scleroderma Spectrum Disorders: A Single Center Experience

    Meredith Rae, Tam Doan and Eyal Muscal, Baylor College of Medicine, Houston, TX

    Background/Purpose: Cardiac disease is a major cause of morbidity and mortality in children with SLE and SSc. Various studies have demonstrated an association between duration…
  • Abstract Number: 086 • 2023 Pediatric Rheumatology Symposium

    Systemic Sclerosis Overlap Syndrome: A Case Series from a Single Large Pediatric Center

    Jessica Nguyen1, Miriah Gillispie-Taylor1, Eyal Muscal1 and Marietta Deguzman2, 1Baylor College of Medicine, Houston, TX, 2Baylor College of Medicine, Texas Children's Hospital, Houston, TX

    Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare but potentially life-threatening autoimmune condition with features including immune, fibrotic, and vascular manifestations affecting the skin and…
  • Abstract Number: 098 • 2023 Pediatric Rheumatology Symposium

    Characterization of Pulmonary Nodules in Juvenile-onset Systemic Sclerosis: A Single Center Case-Series

    Jonathan Li1, Franziska Rosser1, Sameh Tadros1 and Kathryn Torok2, 1UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, 2University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Given the high prevalence of intestinal lung disease (ILD) in systemic sclerosis (SSc) and the low sensitivity of pulmonary function testing for diagnosing ILD,…
  • Abstract Number: 116 • 2023 Pediatric Rheumatology Symposium

    Clinical Significance of Anti-Scl-70 Antibodies in Pediatric Lupus Patients: A Single Center Cohort

    Dawn Janysek1, Yiressy Pina2, Danielle Guffey3 and Marietta De Guzman1, 1Baylor College of Medicine, Texas Children's Hospital, Houston, TX, 2Baylor College of Medicine, Texas Children's Hospital, Houston, TX, 3Baylor College of Medicine, Houston, TX

    Background/Purpose: Production of autoantibodies is a hallmark of SLE, with ANAs as a required diagnostic feature and anti-dsDNA and anti-Smith antibodies being disease-specific. Anti-Scl-70 antibodies…
  • Abstract Number: 123 • 2023 Pediatric Rheumatology Symposium

    Development of Specific Classification Criteria for Juvenile System Sclerosis Patients: A Scoping Review

    Ioana Dobre1, Suzanne Li2, Natalia Vasquez Canizares3, Barbara Reich4, Xurong Zhao5, Quinn McCormick6 and Marinka Twilt5, 1Alberta Children's Hospital/University of Calgary, Calgary, AB, Canada, 2Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ, 3Children's Hospital at Montefiore, New York, NY, 4Hackensack University Medical Center, Hackensack, NJ, 5Alberta Children's Hospital, Calgary, AB, Canada, 6Hackensack Medical Hospital Network, Hackensack, NJ

    Background/Purpose: Juvenile systemic sclerosis (jSSc) is associated with one of the highest morbidity and mortality rate in pediatric rheumatology, yet care recommendations are based upon…
  • Abstract Number: 1052 • ACR Convergence 2022

    Short-term Safety of COVID-19 Vaccination in Systemic Sclerosis Patients: Report from a Global Patient-Reported E-survey

    Darpan Thakare1, Naveen R1, John Pauling2, Sakir Ahmed3, Chris Wincup4, Nicolletta Del Papa5, Gianluca Sambataro6, Fabiola Atzeni7, Marcello Govoni8, SIMONE PARISI9, Elena Bartoloni Bocci10, Gian Domenico Sebastiani11, Enrico Fusaro12, Marco Sebastiani13, Luca Quartuccio14, Franco Franceschini15, Pier Paolo Sainaghi16, Giovanni Orsolini17, Rossella De Angelis18, Maria Giovanna Danielli19, Vincenzo Venerito20, Parikshit Sen21, Minchul Kim22, Abraham Edgar Gracia-Ramos23, Akira Yoshida24, James B. Lilleker25, Vishwesh Agarwal26, Sinan Kardes27, Jessica Day28, Marcin Milchert29, Mrudula Joshi30, Tamer A Gheita31, Babur Salim32, Ioannis Parodis33, Albert Selva O’Callaghan34, Elena Nikiphorou35, Tulika Chatterjee22, Ai Lyn Tan36, Arvind Nune37, Lorenzo Cavagna38, Samuel Shinjo39, Nelly Ziade40, Johannes Knitza41, Hector Chinoy42, Oliver Distler43, Masataka Kuwana44, Rohit Aggarwal45, Latika Gupta46, Vikas Agarwal1 and Ashima Makol47, 1Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, India, 2North Bristol NHS Trust, Bristol, United Kingdom, 3Kalinga Institute of Medical Sciences, Bhubaneswar, India, 4Rayne Institute, University College London, London, United Kingdom, 5Unità operativa complessa (UOC) Day Hospital Reumatologia via Gaetano Pini 9, Centro Specialistico Ortopedico Traumatologico, Gaetano Pini-CTO,, Milano, 6Medico Immunologia e reumatologia presso, Artoreuma S.R.L., Cors S, Mascalucia, 7Rheumatology Unit, University of Messina, Messina, Italy, 8S. Anna Hospital and University of Ferrara, Ferrara, Italy, 9Italian Society for Rheumatology, Turin, Italy, 10Rheumatology Unit. Department of Medicine, Perugia, Perugia, Italy, 11U.O.C. Reumatologia, Ospedale San Camillo-Forlanini,, Roma, 12Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino, Turin, Italy, 13Azienda Policlinico di Modena, Modena, Italy, 14Department of Medicine (DAME), ASUFC, University of Udine, Udine, 15Department of Clinical and Experimental Sciences, ASST Spedali Civili and University of Brescia, Brescia, Italy, 16IRCAD, Interdisciplinary Research Center of Autoimmune Diseases, Novara, Italy, 17Department of Medicine, Rheumatology Unit, University of Verona, Verona, Verona, 18Rheumatology Unit, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Marche, Italy, 19Dipartimento di Scienze Cliniche e Molecolari, Università Politecnica delle, Marche, Italy, 20Department of Emergency and Organ Transplantations-Rheumatology Unit, University of Bari "Aldo Moro", Bari, Italy, 21Maulana Azad Medical College, New Delhi, India, 22University of Illinois College of Medicine Peoria, Peoria, IL, 23Instituto Mexicano del Seguro Social, Ciudad de México, Mexico, 24Nippon Medical School Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan, 25The University of Manchester, Manchester, United Kingdom, 26Mahatma Gandhi Missions Medical College, Lucknow, India, 27Istanbul University, Istanbul, Turkey, 28Walter and Eliza Hall Institute, Melbourne, Australia, 29Pomeranian Medical University in Szczecin, Szczecin, Poland, 30Byramjee Jeejeebhoy Government Medical College and Sassoon General Hospitals, Pune, India, 31Rheumatology Department, Faculty of Medicine, Cairo University, Cairo, Egypt, 32Fauji foundation hospital Rawalpindi, Rawalpindi, Pakistan, 33Karolinska Institutet, Stockholm, Sweden, 34Hospital Universitari Vall d'Hebron, Barcelona, Spain, 35Leiden University Medical Center & King's College London, London, United Kingdom, 36University of Leeds, Leeds, United Kingdom, 37Southport and Ormskirk Hospital NHS Trust, Southport, United Kingdom, 38Università di Pavia, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy, Pavia, Italy, 39Faculdade de Medicina FMUSP, Universidade de São Paulo, São Paulo, Brazil, 40Saint-Joseph University, Beirut, Lebanon, 41Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg and Universitätsklinikum Erlangen, 91054 Erlangen, Germany; Deutsches Zentrum Immuntherapie, Friedrich-Alexander-UniversityErlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, 42The University of Manchester, Sale, United Kingdom, 43Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 44Nippon Medical School Graduate School of Medicine, Tokyo, Japan, 45Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, 46Royal Wolverhampton Trust, Wolverhampton/University of Manchester, United Kingdom, 47Mayo Clinic, Rochester, MN, Rochester, MN

    Background/Purpose: Dedicated data on COVID-19 vaccine-related adverse events (ADEs) in patients with systemic sclerosis (SSc) is limited. Therefore, we aimed to compare all patient-reported ADEs…
  • Abstract Number: 1181 • ACR Convergence 2022

    Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis

    Feiyang Ma1, Pei-Suen Tsou1, Grace Hile1, John Varga1, J. Michelle Kahlenberg1, Allison Billi1, Johann Gudjonsson1 and Dinesh Khanna2, 1University of Michigan, Ann Arbor, MI, 2Division of Rheumatology, Department of Internal Medicine, Scleroderma Program, University of Michigan, Ann Arbor, MI

    Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…
  • Abstract Number: 1622 • ACR Convergence 2022

    TNF Receptor 1 Drives Murine Pulmonary Arterial Hypertension and Is Characterized by Loss of Capillary Endothelial Cells and Pericytes, Smooth Muscle Cell Proliferation, and Alterations in Fibroblast Phenotype

    Stacey Duemmel1, Marc Nuzzo1, Soumyaroop Bhattacharya1, Qingfu Xu1, Amy Mohan1 and Benjamin Korman2, 1URMC, Rochester, NY, 2University of Rochester, Rochester, NY

    Background/Purpose: We previously demonstrated that TNF-transgenic (TNF-Tg) mice have findings consistent with connective-tissue disease associated pulmonary arterial hypertension (CTD-PAH), and that this pathology is mediated…
  • Abstract Number: 2171 • ACR Convergence 2022

    Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous Systemic Sclerosis

    Ranjitha Karanth1, Giuseppina Abignano2, Vishal Kakkar1, Rebecca Ross1, Chris Denton3 and Francesco Del Galdo4, 1University of Leeds, Leeds, United Kingdom, 2Istituto Reumatologico Lucano (IReL), Azienda Ospedaliera Regionale San Carlo, Potenza, Italy, 3University College London, London, United Kingdom, 4Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom

    Background/Purpose: Type-I Interferon pathway activation has been associated with severity and progression of diffuse cutaneous SSc (dcSSc). The role of type-I Interferons (IFNs) in limited…
  • Abstract Number: 1054 • ACR Convergence 2022

    Distinct Differences in Complement Perturbation Between Scleroderma-related Interstitial Lung Disease with and Without Emphysema

    Claire Beesley1, Alice Cole2, Nina Goldman1, Odewale Oluwakorede1, Joseph Barnett2, David Abraham1, Chris Denton1, Rizgar Mageed3 and Voon Ong4, 1University College London, London, United Kingdom, 2Royal Free Hospital, London, United Kingdom, 3Experimental Medicine and Rheumatology, William Harvey Research Institute, Queen Mary University, London, United Kingdom, 4UCL Medical School Royal Free Campus, London, United Kingdom

    Background/Purpose: Aberrant complement activation is associated with autoimmune diseases including systemic sclerosis (SSc). As a leading cause of death in SSc, interstitial lung disease (ILD)…
  • Abstract Number: 1183 • ACR Convergence 2022

    Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq

    Kristina Clark1, Alice Cole2, Shiwen Xu1, Voon Ong3, Christopher Buckley4 and Chris Denton1, 1University College London, London, United Kingdom, 2Royal Free Hospital, London, United Kingdom, 3UCL Medical School Royal Free Campus, London, United Kingdom, 4University of Oxford, Oxford, United Kingdom

    Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…
  • Abstract Number: 1657 • ACR Convergence 2022

    Extreme Phenotype Approach Identifies Rare Variants in Systemic Sclerosis and Dermatomyositis Patients with Severe Calcinosis

    Srijana Davuluri1, Urvashi Kaundal2, Christian Lood3, Puneet Kapoor1, Yumeko Kawano4, Stefania Dell'Orso5, Zuoming Deng6, Zsuzsanna McMahan7, Ami Shah7, Laura Hummers8, Daniel Kastner9, Fredrick Wigley10, David Fiorentino11, Pravitt Gourh12 and Lorinda Chung11, 1Stanford University School of Medicine, Palo Alto, CA, 2National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 3Division of Rheumatology, University of Washington, Seattle, WA, 4Brigham and Women's Hospital, Boston, MA, 5NIH, Bethesda, MD, 6National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, 7Johns Hopkins Rheumatology, Baltimore, MD, 8Johns Hopkins Univerisity, Baltimore, MD, 9National Human Genome Research Institute, Bethesda, MD, 10Johns Hopkins University, Baltimore, MD, 11Stanford University, Stanford, CA, 12National Institutes of Health, Bethesda, MD

    Background/Purpose: Calcinosis, deposition of insoluble calcium salts in skin and subcutaneous tissues, affects up to 40% of systemic sclerosis (SSc) patients, and up to 20%…
  • Abstract Number: 2173 • ACR Convergence 2022

    Rheumatology Telemedicine Consults: Impact of Demographic Variability on Patient Perception in Systemic Sclerosis versus Rheumatoid Arthritis Pts

    Yossra Suliman1, Julia mumper2, Gary Feldman3, M. Cameron Hay4 and Daniel Furst5, 1Assiut University Hospital, Assiut, Egypt, 2Miami University, Miami, OH, 3Pacific Arthritis, Los Angeles, CA, 4Miami University (Ohio), Oxford, OH, 5University of California Los Angeles, Los Angeles, CA

    Background/Purpose: In the era of emerging need to utilize remote technology-based health care services due to the COVID-19 pandemic, we wanted to evaluate patient satisfaction…
  • Abstract Number: 1055 • ACR Convergence 2022

    Associations of Potential Risk Factors with Severe Outcomes in Scleroderma Renal Crisis: Results from a Single Center Study

    Alex Luta1, Saloni Godbole1 and Virginia Steen2, 1Medstar Georgetown University Hospital, Washington, DC, 2Georgetown University School of Medicine, Washington, DC

    Background/Purpose: Scleroderma Renal Crisis (SRC) is a severe and life-threatening complication of Systemic Sclerosis (SSc). Given the rare nature of SSc, it is challenging to…
  • Abstract Number: 1184 • ACR Convergence 2022

    Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II

    Sirapa Vichaikul1, William Brodie2, Megan Mattichak2, Qi Wu2, Dinesh Khanna2 and Eliza Pei-Suen Tsou2, 1Michigan Medicine, Howell, MI, 2University of Michigan, Ann Arbor, MI

    Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…
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