Abstracts tagged "Scleroderma"

Abstract Number: 0694 • ACR Convergence 2024
A Unified Vascular Phenotype Score Identifies Systemic Sclerosis Endotypes and Predicts Prognostic Outcomes: Results from the EUSTAR Database
Stefano Di Donato¹, Michael Hughes², John Pauling³, Marco Matucci-Cerinic⁴, Lesley-Anne Bissell⁵, Edward Jude⁶, Christopher Denton⁷, Yannick Allanore⁸, Marie-Elise Truchetet⁹ and Francesco Del Galdo¹⁰, and EUSTAR collaborators, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ²Tameside and Glossop Integrated NHS Foundation Trust & The University of Manchester, Manchester, United Kingdom, ³North Bristol NHS Trust, Bristol, United Kingdom, ⁴University San Raffaele Milano, Milano, Milan, Italy, ⁵Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁶Tameside and Glossop Integrated Care NHS Foundation Trust, Ashton-under-Lyne, United Kingdom, ⁷University College London, Northwood, United Kingdom, ⁸Université Paris Cité, Paris, France, ⁹Bordeaux University Hospital, Bordeaux, France, ¹⁰University of Leeds, Leeds, United Kingdom
Background/Purpose: Vascular dysfunction is pivotal in Systemic Sclerosis (SSc) pathogenesis, leading to somatic vascular signs such as pitting scars, Digital Ulcers (DUs), telangiectasia as well…
Abstract Number: 0964 • ACR Convergence 2024
Engaging the PD-1 Pathway Attenuates Inflammation Associated Fibrosis in Systemic Sclerosis Fibroblasts and a Preclinical Mouse Model
Maithri Aspari¹, Voon Ong², Klaus Soendergaard³, Esben Naeser⁴, Malene Hvid⁴, Angela Tam⁵, Shiwen Xu⁵, Christopher Denton⁶, David Abraham⁷, Bent Deleuran¹ and Stinne Greisen⁸, ¹Aarhus University, Aarhus, Denmark, ²University College London, London, England, United Kingdom, ³Aarhus University Hospital, Aarhus, Denmark, ⁴AARHUS UNIVERSITET, AARHUS C, Denmark, ⁵University College London, London, United Kingdom, ⁶University College London, Northwood, United Kingdom, ⁷UCL, London, United Kingdom, ⁸Aarhus University/Aarhus University Hospital, Aarhus, Denmark
Background/Purpose: The precise molecular mechanisms driving fibrosis in diffuse cutaneous systemic sclerosis (dcSSc) remain to be elucidated. The immune regulatory programmed cell death protein 1…
Abstract Number: 1573 • ACR Convergence 2024
Early Scleroderma with Non-Raynaud’s Symptoms Prior to Raynaud’s Onset Is Associated with Rapid Progression to Diffuse Skin Disease and Joint Contractures
Iqtidar Hanif¹, Shervin Assassi¹, Maureen Mayes¹, Meng Zhang¹, Julio Charles¹, John VanBuren², Jessica Alvey², Kimia Ghaffari², Elana Bernstein³, Flavia Castelino⁴, Lorinda Chung⁵, Luke Evnin⁶, Tracy Frech⁷, Jessica Gordon⁸, Faye Hant⁹, Laura Hummers¹⁰, Dinesh Khanna¹¹, Kimberly Lakin¹², Dorota Lebiedz-Odrobina¹³, Yiming Luo¹⁴, Ashima Makol¹⁵, Jerry Molitor¹⁶, Duncan Moore¹⁷, Carrie Richardson¹⁸, Nora Sandorfi¹⁹, Ami Shah²⁰, Ankoor Shah²¹, Victoria Shanmugam²², Virginia Steen²³, Elizabeth Volkmann²⁴, Carleigh Zahn¹¹ and Brian Skaug¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²University of Utah, Salt Lake City, UT, ³Division of Rheumatology, Department of Medicine, Vagelos College of Physicians and Surgeons, Columbia University Irving Medical Center, New York, NY, ⁴Massachusetts General Hospital, Boston, MA, ⁵Stanford University, Woodside, CA, ⁶Scleroderma Research Foundation, Brisbane, CA, ⁷Vanderbilt University Medical Center, Nashville, TN, ⁸Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁹Medical University of South Carolina, Charleston, SC, ¹⁰Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ¹¹University of Michigan, Ann Arbor, MI, ¹²Hospital for Special Surgery, New York, NY, ¹³University of Utah, Cottonwood Heights, UT, ¹⁴Columbia University, New York, NY, ¹⁵Mayo Clinic, Rochester, MN, Rochester, MN, 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, ²²Office of Autoimmune Disease Research, Office of Research on Women's Health, National Institutes of Health, Great Falls, VA, ²³Georgetown University School of Medicine, Washington, DC, ²⁴University of California, Department of Medicine, Los Angeles, CA, USA, Los Angeles
Background/Purpose: Raynaud’s phenomenon (RP) is often the initial clinical manifestation of systemic sclerosis (SSc). RP alerts clinicians to the possibility of an autoimmune rheumatic disease,…
Abstract Number: 1826 • ACR Convergence 2024
Unveiling the Primary Cilia Signature Driving Systemic Sclerosis Pathogenesis
Le My Tu Nguyen¹, Carlos Córdova-Fletes², Poulami Dey³, Johann Gudjonsson³, Rebecca Wells⁴, Rebecca Ross⁵, Natalia Riobo-Del Galdo⁴, Francesco Del Galdo⁴, Dibyendu Bhattacharyya³, John Varga³ and Maria Teves¹, ¹Virginia Commonwealth University, Richmond, VA, ²Universidad Autónoma de Nuevo León, Monterrey, Mexico, ³University of Michigan, Ann Arbor, MI, ⁴University of Leeds, Leeds, United Kingdom, ⁵Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Fibrosis in multiple organs is the defining hallmark that accounts for the high mortality associated with systemic sclerosis (SSc). Understanding how SSc patients develop…
Abstract Number: 2453 • ACR Convergence 2024
Gastrointestinal Symptom Severity and Intestinal Permeability in Systemic Sclerosis: A Single Center Prospective Study
Elvira Lesmana¹, Ashley Keehn², Anukul Karn³, Andrea Pauly⁴, Margaret Breen-Lyles², Adam L Edwinson², Madhusudan Grover⁵ and Ashima Makol⁵, ¹Mayo Clinic, Rochester, MN, New York, NY, ²Mayo Clinic, Rochester, MN, Rochester, ³Mayo Clinic, Rochester, MN, Farmington Hills, MI, ⁴Mayo Clinic Rochester, Rochester, MN, ⁵Mayo Clinic, Rochester, MN, Rochester, MN
Background/Purpose: Systemic Sclerosis (SSc) is a complex, heterogeneous, multisystem autoimmune disease with high morbidity and mortality. Gastrointestinal symptoms impact more than 90% of SSc patients,…
Abstract Number: PP11 • ACR Convergence 2024
Living Well with Chronic Illness: Decades with MCTD
Carol KAMINSKI, HP (Hewlett Packard) Retired, Wilmington, NC
Background/Purpose: In the fall of 1985, I was not feeling well. I attributed my tiredness, weakness and malaise to stress and carried on. Walking upstairs I…
Abstract Number: 0695 • ACR Convergence 2024
Prognostic Value of Serum Type I Interferon in Predicting Morbi-Mortality Outcomes in Systemic Sclerosis: Insights from the STRIKE Basket Cohort
Stefano Di Donato¹, Marco Minerba², Enrico De Lorenzis³, Collette Hartley⁴, Lesley-Anne Bissell⁵, Rebecca Ross⁶ and Francesco Del Galdo⁴, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ²Campus Bio-Medico University of Rome, Taranto, United Kingdom, ³Catholic University of the Sacred Heart, Roma, Rome, Italy, ⁴University of Leeds, Leeds, United Kingdom, ⁵Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁶Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) encompasses limited cutaneous (lc)SSc and diffuse cutaneous (dc)SSc, with lcSSc affecting more than 60% of patients and dcSSc associated being burdened…
Abstract Number: 0965 • ACR Convergence 2024
Scleroderma Associated Interstitial Lung Disease Is Characterized by Aberrant Lung Epithelial Remodeling
Monica Yang¹, Fred Deiter², Emily Flynn², Seoyeon Lee³, Jessica Neely¹, John Greenland², Marina Sirota² and Paul Wolters², ¹UCSF, San Francisco, CA, ²UCSF, SF, ³Yale, New Haven
Background/Purpose: Interstitial lung disease (ILD) is present in a majority of systemic sclerosis (SSc) patients and the leading cause of SSc-related mortality. A subset of…
Abstract Number: 1577 • ACR Convergence 2024
Elevated Neutrophil Extracellular Traps in Systemic Sclerosis-associated Vasculopathy and Suppression by a Synthetic Prostacyclin Analog
Neda Kortam¹, Wenying Liang¹, Claire Shiple¹, Suiyuan Huang¹, Rosemary Gedert¹, James St. Clair¹, Cyrus Sarosh², Caroline Foster¹, Eliza Pei-Suen Tsou¹, John Varga¹, Jason Knight¹, Dinesh Khanna¹ and Ramadan Ali¹, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan, Temperance, MI
Background/Purpose: Neutrophils and neutrophil extracellular traps (NETs) contribute to the vascular complications of multiple diseases, but their role in systemic sclerosis (SSc) is understudied. We…
Abstract Number: 1829 • ACR Convergence 2024
Genomic Analysis of Skin Biopsies Differentiates Major Anti-Nuclear Autoantibody Subsets in Limited Cutaneous Systemic Sclerosis
Philip Yee¹, Medha Kanitkar², Kristina Clark³, Voon Ong¹ and Christopher Denton⁴, ¹University College London, London, England, United Kingdom, ²University College London, London, United Kingdom, ³University of Oxford, Oxford, United Kingdom, ⁴University College London, Northwood, United Kingdom
Background/Purpose: Systemic sclerosis (SSc) displays significant heterogeneity. Those with limited cutaneous systemic sclerosis (lcSSc) experience significant systemic organ involvement similar to those with diffuse cutaneous…
Abstract Number: 2454 • ACR Convergence 2024
Age-Adjusted Mortality Rate Trends in Systemic Sclerosis in the United States from 1999 to 2020
Ansaam Daoud¹, Loai Dweik², Muhammad Shamim³, Haseeb Chaudhary⁴ and Omer Pamuk⁵, ¹Case Western Reserve University/University Hospitals, Akron, OH, ²Cleveland Clinic Akron General Internal Medicine, Akron, OH, ³University Hospitals/ Case Western Reserve University, Cuyahoga Falls, OH, ⁴Case Western Reserve University, Westlake, OH, ⁵University Hospitals Cleveland Medical Center/ Case Western Reserve University, Cleveland, OH
Background/Purpose: Of all rheumatic diseases, systemic sclerosis (SSc) has the highest disease-specific mortality, with a higher mortality risk than the general population. North America has…
Abstract Number: 0696 • ACR Convergence 2024
MRI DAVIX Index Is an Imaging Biomarker for Endothelial Damage of Systemic Sclerosis Digital Ulcers
Stefano Di Donato¹, Rebecca Ross², Yassir El-Sherbiny³, Riccardo Bixio⁴, Marco Minerba⁵, Christopher Wasson⁶, Jun Li⁷, Jochen SCHMITZ⁸, Sudha Visvanathan⁹ and Francesco Del Galdo⁶, ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, Chapel Allerton Hospital, Leeds, UK, Leeds, United Kingdom, ²Medicine and Health, University of Leeds, Leeds, United Kingdom, ³Nottingham Trent University, Nottingham, United Kingdom, ⁴University of Verona, Verona, Verona, Italy, ⁵1. Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, 2. NIHR Leeds Biomedical Research Centre, United Kingdom, Leeds, United Kingdom, ⁶University of Leeds, Leeds, United Kingdom, ⁷Boehringer Ingelheim, Danbury, CT, ⁸Boehringer-Ingelheim, Ridgefield, CT, ⁹Boehringer-Ingelheim Pharmaceuticals, Ridgefield, CT
Background/Purpose: Circulating endothelial cells (CEC) and their precursors have been shown to correlate to the severity of vascular manifestations in Systemic sclerosis1, such as Digital…
Abstract Number: 0970 • ACR Convergence 2024
Deubiquitylase (DUB) BRCC36 Isopeptidase Complex (BRISC) Inhibitors Prevent IFNAR1 Deubiquitination to Restore Natural Type I IFN Response in Autoimmunity
Rebecca Ross¹, Poli Adi Narayanna Reddy², Joseph Salvino², Elton Zeqiraj³ and Francesco Del Galdo³, ¹Medicine and Health, University of Leeds, Leeds, United Kingdom, ²The Wistar Institute, Philadelphia, PA, ³University of Leeds, Leeds, United Kingdom
Background/Purpose: Increased Type I IFN activation plays a key role in Scleroderma (SSc), Systemic Lupus Erythematosus and Inflammatory Myositis. Deubiquitylase (DUB) BRCC36 isopeptidase complex (BRISC)…
Abstract Number: 1583 • ACR Convergence 2024
The Association of Weakpositive Anti-RNA Polymerase III Antibodies with the Diagnosis and Complications of Systemic Sclerosis and Other Systemic Autoimmune Diseases
Mashood Badshah¹, Warren Balaja², Brannon Donovan³, Paul Schmidt³ and Megan Krause⁴, ¹The University of Kansas Medical Center, Kansas City, KS, ²University of Kansas Medical Center, Kansas City, KS, ³University of Kansas Medical Center, Kansas City, ⁴University of Kansas, Kansas City, KS
Background/Purpose: Anti-RNA polymerase III antibody (ARA) is associated with systemic sclerosis (SSc). The presence of ARA in patients with SSc is associated with the risk…
Abstract Number: 1830 • ACR Convergence 2024
Single-cell RNA Sequencing Profiling of Very Early-Stage Systemic Sclerosis Skin Reveals a Fibroblast Pro-inflammatory Gene Signature and Keratinocyte Dysregulation
Lumeng Li¹, Elena Pachera², Rucsandra Dobrota³, Kristina Bürki¹, Carina Mihai³, Muriel Elhai⁴, Laura Much¹, Astrid Hofman¹, Pietro Bearzi⁵, Sophie Wagner⁶, Sarah Lötscher⁷, Anna-Maria Hoffmann-Vold⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁴University Hospital zurich, Zürich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁶University of Zurich, Schlieren, Switzerland, ⁷Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Zurich, Switzerland, ⁸Oslo University Hospital, Oslo, Norway, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: The poor prognosis of patients with systemic sclerosis (SSc) underscores the need to prevent disease onset and irreversible organ damage. This study aims to…

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