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Abstracts tagged "Scleroderma, Systemic"

  • 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: L06 • ACR Convergence 2023

    Improvement in Clinical and Patient-Reported Outcomes for Refractory Juvenile-Onset Systemic Sclerosis (jSSc) 6 Months to 2 Years After Autologous Stem Cell Transplantation (ASCT)

    Kathryn Torok1, Paulina Horvei1, Franziska Rosser1, Kirsten Rose-felker2, Vibha Sood2, Adam Olsen2, Nicole Hogue2, Vickie Vandergrift2, Lauren Farver2, Devin Mcguire2, Jonathan Li3, Haley Havrilla2, Jessie Alexander4, Shawna McIntyre2 and Paul Szabolcs1, 1University of Pittsburgh; UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, 2UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, 3University of Pittsburgh Medical Center, Pittsburgh, PA, 4Stanford Children's Hospital, Palo Alto, CA

    Background/Purpose: Juvenile-onset systemic sclerosis (jSSc) is an inflammatory, fibrotic, and vasculopathic disease that causes severe multi-organ dysfunction leading to significant morbidity and early mortality.When patients…
  • Abstract Number: 0614 • ACR Convergence 2023

    Self-Reported Sexual Dysfunction and Perceptions of Rheumatologist Engagement on This Issue in Patients with Systemic Sclerosis

    Liza Morales1, Robert Spiera2, Jessica Gordon1, Deanna Jannat-Khah1 and Kimberly Lakin1, 1Hospital for Special Surgery, New York, NY, 2Hosptial for Special Surgery, New York, NY

    Background/Purpose: Few studies have investigated the prevalence and risk factors of sexual dysfunction among individuals with systemic sclerosis (SSc). The purpose of this study is…
  • Abstract Number: 1523 • ACR Convergence 2023

    The Risk of Scleroderma Renal Crisis from Intraarticular Corticosteroid Injection in Systemic Sclerosis

    Muruganandam Maheswari, Eyerusalem Akpan, Matthew Mcelwee, Meredith Keller, Angie Ariza - Hutchinson, Rosemina Patel, Wilmer Sibbitt, Frank O'Sullivan, Sharon nunez, N. Suzanne Emil and Roderick Fields, University of New Mexico, Albuquerque, NM

    Background/Purpose: Scleroderma renal crisis (SRC) is a rare but a life-threatening complication of systemic sclerosis (SSc), affecting 2-15% of patients with SSc. SRC has been…
  • Abstract Number: 0628 • ACR Convergence 2023

    Interstitial Lung Disease in Very Early Systemic Sclerosis

    Sinziana Muraru1, Carina Mihai1, Anna-Maria Hoffmann-Vold2, Cosimo Bruni1, Muriel Elhai3, Mike Becker4, Suzana Jordan1, Alexandru Garaiman1, Oliver Distler1 and Rucsandra Dobrota1, 1Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, 2Oslo University Hospital, Oslo, Norway, 3Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, 4Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland

    Background/Purpose: Interstitial lung disease (ILD) affects around 50% of patients with systemic sclerosis (SSc) and is the main cause of mortality. Preliminary data from small…
  • Abstract Number: 1526 • ACR Convergence 2023

    Evusheld Efficacy and Safety in Autoimmune Rheumatic Diseases (AIRD) Treated by B Cell Depeting Therapy or Autologous Stem Cell Transplantation: A Prospective Observational Study

    Lisa Kaly1, mark Volevich1, shiri keret2, Abid Awisat3, Aniela Shouval1, Itzhak Rosner4, Michel Rozenbaum1, Nina Boulman1 and Doron Rimar1, 1Bnai Zion Medical Center, Haifa, Israel, 2Bnai Zion, Atlit, Israel, 3Bney-Zion Medical Center, Baqa Elgharbiya, Israel, 4Bnai Zion Medical Center/Technion, Haifa, Israel

    Background/Purpose: B cell depleting therapy (rituximab) and Autologous Hematopoietic Stem Cell transplantation (AHSCT) result in impaired ability to mount a humoral immune response. Evusheld (tixagevimab…
  • Abstract Number: 0630 • ACR Convergence 2023

    Prevalence and Risk Factors for Systemic Sclerosis Digital Ischemic Complications in the Collaborative National Quality and Efficacy Registry

    Marissa Savoie1, Monica Harding2, Jessica Alvey2, John VanBuren2, Shervin Assassi3, Elana Bernstein4, Lorinda Chung5, Luke Evnin6, Tracy Frech7, Jessica Gordon8, Faye Hant9, Laura Hummers10, Dinesh Khanna11, Kimberly Lakin8, Dorota Lebiedz-Odrobina12, Yiming Luo13, Ashima Makol14, Jerry Molitor15, Duncan Moore16, Carrie Richardson17, Nora Sandorfi18, Ami Shah19, Ankoor Shah20, Brian Skaug21, Virginia Steen22, Elizabeth Volkmann23 and Flavia Castelino1, 1Massachusetts General Hospital, Boston, MA, 2University of Utah, Salt Lake City, UT, 3University of Texas McGovern Medical School at Houston, Houston, TX, 4Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, 5Department of Medicine, Division of Immunology and Rheumatology, Stanford University School of Medicine, Woodside, CA, 6Scleroderma Research Foundation, Brisbane, CA, 7Vanderbilt University Medical Center, Nashville, TN, 8Hospital for Special Surgery, New York, NY, 9Medical University of South Carolina, Charleston, SC, 10Johns Hopkins University, Baltimore, MD, 11University of Michigan, Ann Arbor, MI, 12University of Utah, Cottonwood Heights, UT, 13Division of Rheumatology, Department of Medicine, Columbia University Irving Medical Center, New York, NY, 14Mayo Clinic, Rochester, MN, Rochester, MN, 15University of Minnesota, Minneapolis, MN, 16Northwestern University, Department of Rheumatology, Chicago, IL, 17Northwestern University, Chicago, IL, 18University of Pennsylvania, Philadelphia, PA, 19Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Ellicott City, MD, 20Duke University, Durham, NC, 21Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX, 22Georgetown University School of Medicine, Washington, DC, 23University of California Los Angeles, Los Angeles, CA

    Background/Purpose: Digital ischemic ulcers (DIU) develop in 36-44% of patients with systemic sclerosis (SSc).1,2Prior international observational studies have evaluated characteristics of digital pitting and ulcers…
  • Abstract Number: 1528 • ACR Convergence 2023

    Intravenous or Subcutaneous Immunoglobulins as Potential Treatment for Gastrointestinal, Cutaneous and Vascular Involvement in Systemic Sclerosis: Data from an Italian Cohort of 65 Patients

    Francesco Bonomi1, Arianna Damiani1, Carmela Coccia1, Juela Levani1, elisa Fiorentini2, silvia peretti1, Gemma Lepri1, martina orlandi1, francesca bartoli1, marco Matucci Cerinic3, silvia bellando randone1 and serena guiducci1, 1Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Florence, Italy, 2Department of Experimental and Clinical medicine, Division of Rheumatology, Azienda Ospedaliero Universitaria Careggi, University of Florence, Firenze, Italy, 3Unit of Immunology, Rheumatology, Allergy and Rare diseases, IRCCS San Raffaele Hospital, Milan, Milan, Italy

    Background/Purpose: Treatment of systemic sclerosis (SSc) is challenging, and in the last 2 decades there has been an increasing interest for immunoglobulin (Igs) treatment as…
  • Abstract Number: 0635 • ACR Convergence 2023

    Clinical Characteristics and Longitudinal Outcomes of Patients with Childhood-Onset Systemic Sclerosis at a Tertiary Center

    Jessica Nguyen1, Andrea Ramirez2, Maria Pereira2, Eyal Muscal3, Manuel Silva-Carmona2 and Marietta De Guzman2, 1Baylor College of Medicine, Manvel, TX, 2Baylor College of Medicine, Houston, TX, 3Division of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, TX

    Background/Purpose: Childhood-onset systemic sclerosis (SSc) is a rare autoimmune multisystemic condition with immune, fibrotic, and vascular manifestations. Although progress has been made in recognizing this…
  • Abstract Number: 1663 • ACR Convergence 2023

    Clinically Severe Systemic Sclerosis Skin Harbors Inflammatory Fibroblasts Associated with Lymphocytes and Plasmacytoid Dendritic Cells

    Kimberly Lakin1, Robert Spiera2, Yaxia Zhang1, David Oliver1, Aliza Bloostein1, Hiranmayi Ravichandran3, Niroshana Anandasabapathy3, Franck Barrat1, Jessica Gordon1 and Dana Orange4, 1Hospital for Special Surgery, New York, NY, 2Hosptial for Special Surgery, New York, NY, 3Weill Cornell Medical College, New York, NY, 4The Rockefeller University, New York, NY

    Background/Purpose: We have previously shown that skin fibroblast activation is related to clinical severity and resolves with improvement in diffuse systemic sclerosis (dcSSc). The immune…
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