ACR Meeting Abstracts

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Abstracts tagged "Scleroderma, Systemic"

  • Abstract Number: 007 • 2023 Pediatric Rheumatology Symposium

    Single Cell RNA Sequencing Analysis of the Skin to Evaluate the Effect of Autologous Stem Cell Transplant on Fibroblast Populations in Juvenile Onset Systemic Sclerosis

    Claire Cheng, Giffin Werner, Anwesha Sanyal and Kathryn Torok, University of Pittsburgh, Pittsburgh, PA

    Background/Purpose: Juvenile systemic sclerosis (JSSc) is a rare autoimmune disease associated with high morbidity. Inflammatory driven multi-organ fibrosis is similar to adult-onset SSc, with 40%…
  • 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: 106 • 2023 Pediatric Rheumatology Symposium

    Caregivers’ Perspectives on Barriers to Care in Juvenile Localized and Systemic Scleroderma

    Leigh Stubbs1, Andrew Ferry2, Danielle Guffey1, Christina Loccke3, Erin Moriarty Wade3, Pamela Pour3, Kaveh Ardalan4, Peter Chiraseveenuprapund5, Ingrid Ganske6, Daniel Glaser7, Gloria Higgins8, Nadia Luca9, Katharine Moore10, Vidya Sivaraman11, Katie Stewart1, Natalia Vasquez Canizares12, Raegan Hunt1, Renata Maricevich1, Kathryn Torok13 and Suzanne Li14, 1Baylor College of Medicine, Houston, TX, 2Oregon Health and Science University, Portland, OR, 3n/a, 4Duke University School of Medicine, Durham, NC, 5University of California - San Diego, San Diego, CA, 6Boston Children’s Hospital and Harvard Medical School, Boston, MA, 7Yale University School of Medicine, New Haven, CT, 8Nationwide Childrens Hospital/ The Ohio State University, Columbus, OH, 9University of Calgary, Calgary, AB, Canada, 10University of Colorado / Children's Hospital Colorado, Denver, CO, 11Nationwide Children's Hospital/ The Ohio State University, Columbus, OH, 12Children's Hospital at Montefiore, New York, NY, 13University of Pittsburgh, Pittsburgh, PA, 14Hackensack Meridian School of Medicine, Joseph M. Sanzari Children's Hospital, Hackensack, NJ

    Background/Purpose: Juvenile localized scleroderma (LS) and systemic sclerosis (SSc) are rare rheumatic diseases often associated with severe morbidities. Delays in diagnosis are common, putting children…
  • 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: 1946 • ACR Convergence 2022

    Focused Clinical Correlation of Autoantibody Testing in Juvenile Systemic Sclerosis and Localized Scleroderma Using the Euroimmun™ Scleroderma Disease Profile

    Jonathan Li1, Emily Mirizio2, Katherine Buhler3, Anne Stevens4, May Choi5, Kaila Schollaert-Fitch2, Kathryn Torok2, Christopher Liu2 and Marvin Fritzler3, 1Combined Internal Medicine-Pediatrics Residency Program, Children's Hospital of UPMC, Pittsburgh, PA, 2Pediatric Rheumatology, Children's Hospital of UPMC, Pittsburgh, PA, 3University of Calgary, Calgary, AB, Canada, 4Janssen, Hansville, WA, 5Brigham and Women's Hospital | University of Calgary, Calgary, AB, Canada

    Background/Purpose: Commercially available autoantibody (AAb) panels are developed for adult rheumatological diseases; application to pediatric disease has not been established. As a pediatric scleroderma referral…
  • Abstract Number: 2155 • ACR Convergence 2022

    Sclerodermoid Reaction Following Immune Checkpoint Inhibitor Therapy: A Case Series from MD Anderson Cancer Center

    Julia Chernis1 and Maryam Buni2, 1McGovern Medical School at the UTHSC Houston, Houston, TX, 2MD Anderson Cancer Center, Bellaire, TX

    Background/Purpose: Immune checkpoint inhibitors (ICIs) have proven to be efficacious in the treatment of multiple malignancies.[1] they are associated immunologic activation has been implicated in…
  • Abstract Number: 2156 • ACR Convergence 2022

    Clinical Benefits of Lung Transplantation in Systemic Sclerosis-related Interstitial Lung Disease; A Single-Center Retrospective Study

    Yoichi Nakayama1, Ran Nakashima1, Tomohiro Handa2, Kiminobu Tanizawa2, Akihiro Ohsumi3, Mirei Shirakashi1, Ryosuke Hiwa1, Hideaki Tsuji1, Koji Kitagori1, Shuji Akizuki4, Hajime Yoshifuji5, Hiroshi date3 and Akio Morinobu5, 1Kyoto University Graduate School of Medicine, Department of Rheumatology and Clinical Immunology, Kyoto, Japan, 2Kyoto University Graduate School of Medicine, Department of Respiratory Medicine, Kyoto, Japan, 3Kyoto University Graduate School of Medicine, Department of Thoracic Surgery, Kyoto, Japan, 4Kyoto universtiy hospital, Kyoto City, Japan, 5Kyoto University, Kyoto, Japan

    Background/Purpose: Systemic sclerosis (SSc) is a chronic autoimmune disorder with microangiopathy and fibrosis affecting the skin and visceral organs. Interstitial lung disease (ILD) is a…
  • Abstract Number: 0435 • ACR Convergence 2022

    Onset of ANCA-associated Vasculitides in Systemic Sclerosis: Phenotype, Management and Outcomes

    deborah Eshagh1, Thomas Quéméneur2, Alexandre Karras3, Viviane Queyrel4, Jean Francois Augusto5, Christian Agard6, Vincent Audard7, Marion Couderc8, PIerre Duffau9, Cecile-Audrey Durel10, Stan Faguer11, Noemie Jourde Chiche12, Aurelie Lavergne13, Christian Lavigne5, Nicolas Limal1, Amélie Servettaz14, Perrine Smets15, Alexis Regent16, Luc Mouthon16 and Benjamin Terrier16, 1APHP, Paris, France, 2Valenciennes Hospital, Valenciennes, France, 3HEGP, Paris, France, 4CHU NIce, Nice, France, 5CHU Angers, Angers, France, 6Internal medicine, Nantes University Hospital, Nantes, France, 7CHU Henri Mondor, Creteil, France, 8University Hospital, Clermont-Ferrand, France, 9CHU Bordeaux, Bordeaux, France, 10CHU Lyon, Lyon, France, 11CHU Toulouse, Toulouse, France, 12APHM, Marseille, France, 13CHU Rennes, Rennes, France, 14CHU Reims, Reims, France, 15CHU Clermont Ferrand, Clermont-Ferrand, France, 16National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France

    Background/Purpose: Systemic sclerosis (SSc) is a complex autoimmune disease characterized by autoantibody production, skin and visceral fibrosis, and vasculopathy. The onset of ANCA-associated vasculitides (AAV)…
  • Abstract Number: 2160 • ACR Convergence 2022

    The Role of CCL21 in Serum Samples from Systemic Sclerosis Patients

    Henriette Didriksen1, Øyvind Molberg2, Adi Mehta3, Suzana Jordan4, Håvard Fretheim3, Einar Gude3, Thor Ueland3, Cathrine Brunborg3, Vyacheslav Palchevskiy5, Torhild Garen3, Øyvind Midtvedt3, Arne Andreassen3, Fridtjof Lund-Johansen3, Oliver Distler4, John Belperio5 and Anna-Maria Hoffmann-Vold3, 1Oslo University Hospital, Moss, Norway, 2Department of Rheumatology, Oslo University Hospital, Oslo, Norway, Oslo, Norway, 3Oslo University Hospital, Oslo, Norway, 4Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 5University of California Los Angeles, Los Angeles, CA

    Background/Purpose: Chemokines appear to be important for the pathogenesis of autoimmune diseases. Humans may generate antibodies targeting chemokines leading to inhibition of signaling and driving…
  • Abstract Number: 0518 • ACR Convergence 2022

    Changes in Skin Fibroblast Polarization Gene Expression Herald Clinical Improvement in Early, Diffuse Cutaneous Systemic Sclerosis

    Kimberly Showalter Lakin1, Deanna Jannat-Khah, DrPH, MSPH1, Yajas Shah2, Olivier Elemento2, Aliza Bloostein1, Jessica Gordon1, Michael Whitfield3, Barbara White4, Dana Orange5 and Robert Spiera1, 1Hospital for Special Surgery, New York, NY, 2Weill Cornell Medicine, New York, NY, 3Dartmouth Geisel School of Medicine, Department of Biomedical Data Science, Lebanon, NH, 4Corbus Pharmaceuticals, Pleasanton, CA, 5The Rockefeller University, New York, NY

    Background/Purpose: We previously identified a group of genes associated with high alpha-smooth muscle actin (aSMA) and low CD34 fibroblast staining in lesional systemic sclerosis (SSc)…
  • Abstract Number: 2162 • ACR Convergence 2022

    Systemic Sclerosis in Alaska Native/American Indian People in Alaska

    Vivek Mehta and Elizabeth Ferucci, Alaska Native Tribal Health Consortium, Anchorage, AK

    Background/Purpose: Systemic sclerosis (SSc) is known to have more severe manifestations and higher mortality in Black populations, while fewer studies have examined disparities in epidemiology…
  • Abstract Number: 1059 • ACR Convergence 2022

    Vision Transformer Assisting Rheumatologists in Screening for Capillaroscopy Changes in Systemic Sclerosis: An Artificial Intelligence Solution

    Alexandru Garaiman1, Farhad Nooralahzadeh2, Carina Mihai1, Nikitas Gkikopoulos1, Nicolas Perez Gonzalez2, Mike Becker1, Oliver Distler1, Michael Krauthammer3 and Britta Maurer4, 1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 2Department of Biomedical Informatics, University of Zurich, Zürich, Switzerland, 3Department of Quantitative Biomedicine, University of Zurich, Zürich, Switzerland, 4Department of Rheumatology and Immunology, University Hospital Bern, University of Bern, Bern, Switzerland

    Background/Purpose: An accurate assessment of nailfold capillaroscopy (NFC) images has great importance in the diagnosis and prognosis of systemic sclerosis (SSc). To overcome some of…
  • Abstract Number: 2165 • ACR Convergence 2022

    Patients with Systemic Sclerosis Have Impaired Forced Vital Capacity, Even in the Absence of Interstitial Lung Disease

    Håvard Fretheim, Imon Barua, Øyvind Midtvedt, Torhild Garen, Phoung Phoung Diep, Michael Durheim, Cathrine Brunborg and Anna-Maria Hoffmann-vold, Oslo University Hospital, Oslo, Norway

    Background/Purpose: Spirometry, in particular forced vital capacity (FVC), is a widely implemented tool in the initial diagnostic workup and monitoring of systemic sclerosis-related interstitial lung…
  • Abstract Number: 1119 • ACR Convergence 2022

    Investigating Macrophage Heterogeneity in the Esophagus and Lungs of SSc Patients

    Hadijat Makinde1, Carla Cuda1, Miranda Gurra1, Mary Carns2, Kathleen Aren2, Gaurav Gadhvi1, Salina Dominguez1, Jane Dematte3, Darren Brenner4, John Pandolfino5, G. R. Scott Budinger4, Deborah Winter6 and Harris Perlman1, 1Northwestern University, Chicago, IL, 2Northwestern University Division of Rheumatology, Chicago, IL, 3Northwestern University, Elmhurst, IL, 4Northwestern University Feinberg School of Medicine, Chicago, IL, 5Northwestern University, Feinberg School of Medicine, Wilmette, IL, 6Northwestern University, Skokie, IL

    Background/Purpose: Our group has made important contributions to an emerging understanding of monocytes and macrophages as central to SSc pathogenesis. There are numerous studies that…
  • Abstract Number: 2174 • ACR Convergence 2022

    Influence of Immunosuppressive Therapy on Gastrointestinal Symptoms in Patients with Systemic Sclerosis

    Lea Stamm1, Alexandru Garaiman1, Norina Zampatti1, Mike Becker1, Cosimo Bruni1, Rucsandra Dobrota1, Muriel Elhai1, Sherif Ismail2, Suzana Jordan1, Aurora Tatu3, Oliver Distler1 and Carina Mihai1, 1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, 2Department of Internal Medicine, National Research Center, Cairo, Egypt, 3Department of Gastroenterology, University Hospital Zurich, University of Zurich, Zürich, Switzerland

    Background/Purpose: The gastrointestinal (GI) tract is frequently affected in systemic sclerosis (SSc), leading to considerable morbidity and even mortality. While important progress has been made…
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