Abstracts tagged "Scleroderma"

Abstract Number: 0679 • ACR Convergence 2024
Comparing Raynaud’s Phenomenon Measurement Tools: Results of the Optimizing Raynaud Phenomenon Outcome Measures in Systemic Sclerosis (ROSS) Study
Rachel Wallwork¹, Haomin Hu², Ami Shah³, Laura Hummers⁴, John Pauling⁵, Victoria Flower⁶, Bambang Parmanto², Andi Saptono² and Robyn Domsic⁷, ¹Johns Hopkins University, Towson, MD, ²University of Pittsburgh, Pittsburgh, PA, ³Division of Rheumatology, Johns Hopkins University, Ellicott City, MD, ⁴Johns Hopkins University, Division of Rheumatology, Baltimore, MD, Ellicott City, MD, ⁵North Bristol NHS Trust, Bristol, United Kingdom, ⁶Royal National Hospital for Rheumatic Diseases, Bath, United Kingdom, ⁷Division of Rheumatology and Clinical Immunology, University of Pittsburgh School of Medicine, Pittsburgh, PA
Background/Purpose: Raynaud’s phenomenon (RP) is the most common symptom in systemic sclerosis (SSc) and can lead to significant morbidity, including digital ulcers and/or gangrene. Despite…
Abstract Number: 0782 • ACR Convergence 2024
30-Color Full Spectrum Flow Cytometry Identifies Sex Differences in the Circulating Immune Landscape of Systemic Sclerosis
Sylvia Posso¹, Jane Buckner², Montserrat Anguera³ and Nikhil Jiwrajka⁴, ¹Benaroya Research Institute at Virginia Mason, Seattle, WA, ²Benaroya Research Institute, Seattle, WA, ³University of Pennsylvania, Philadelphia, PA, ⁴Hospital of the University of Pennsylvania, Philadelphia, PA
Background/Purpose: Systemic sclerosis (SSc) is a heavily female-biased systemic autoimmune disease associated with significant morbidity and mortality due to fibrosis of the skin and vital…
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: 1818 • ACR Convergence 2024
Distribution and Morphological Changes of Adventitial Fibroblasts in Healthy and Scleroderma Skin
Banafsheh Nazari¹, Tracy Tabib², Christina Morse³ and Robert Lafyatis², ¹UPMC-Mercy, Pittsburgh, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pittsburgh, Pittsburgh
Background/Purpose: Our previous single-cell RNA-sequencing study identified two major fibroblast subpopulations in healthy skin biopsies: SFRP2/DPP4 and FMO1/LSP1 fibroblasts¹. Additionally, we discovered that adventitial fibroblasts…
Abstract Number: 1977 • ACR Convergence 2024
Three-dimensional Stereophotogrammetry Has Utility in Tracking Upper Eyelid Involvement and Overall Disease Worsening in Patients with Craniofacial Localized Scleroderma
Tyler Cepica¹, Jennifer Foster², Priya Sarlashkar², Rami Hallac³ and Heidi Jacobe², ¹University of Texas Southwestern Medical School, Benbrook, TX, ²The University of Texas Southwestern Medical Center, Department of Dermatology, Dallas, TX, ³The University of Texas Southwestern Medical Center, Department of Pastic Surgery, Dallas, TX
Background/Purpose: Craniofacial localized scleroderma’s (LoS) indolent and subtle course poses challenges in quantifying disease progression. Traditional serial photography and the Localized Scleroderma Cutaneous Assessment Tool…
Abstract Number: 2643 • ACR Convergence 2024
Integrated Bulk and Single Cell RNA Sequencing Defines Key Pathways Regulating Myofibroblast Differentiation Across ANA Subgroups in Diffuse Systemic Sclerosis
Kristina Clark¹, Corrado Campochiaro², Emma Derrett-Smith³, Voon Ong⁴, Christopher Buckley⁵ and Christopher Denton⁶, ¹University of Oxford, Oxford, United Kingdom, ²IRCCS San Raffaele Hospital. Vita-Salute San Raffaele University, Milan, Milan, Italy, ³University College London Division of Medicine, Birmingham, United Kingdom, ⁴University College London, London, England, United Kingdom, ⁵Kennedy Institute of Rheumatology, University of Oxford, Oxford, United Kingdom, ⁶University College London, Northwood, United Kingdom
Background/Purpose: Myofibroblasts are key cells in the pathogenesis of systemic sclerosis (SSc). TGFβ is a key growth factor driving myofibroblast formation in SSc. The main…
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: 0785 • ACR Convergence 2024
Exploring the Link Between Atgl-Dependent Lipolysis and Dermal Fibrosis in Systemic Sclerosis
Elizabeth Caves¹, Agrani Dixit¹, Anna Jussila², Vivian Lei³, Hailey Edelman⁴, Muhammad Hamdan⁵, Ian Odell⁵, Monique Hinchcliff⁶, Radhika Atit⁷ and Valerie Horsley¹, ¹Yale School of Medicine, New Haven, CT, ²Stanford Medicine, Stanford, CA, ³University of North Carolina School of Medicine, Chapel Hill, ⁴Vanderbilt School of Engineering, New Haven, ⁵Yale School of Medicine, New Haven, ⁶Yale School of Medicine, Westport, CT, ⁷Case Western Reserve University College of Arts and Sciences, Cleveland
Background/Purpose: Resident lipid-filled dermal adipocytes are depleted in both systemic sclerosis (SSc) and scleroderma mouse models, but mechanisms are poorly understood. We undertook studies in mouse…
Abstract Number: 1568 • ACR Convergence 2024
Distribution of Proteinuria in Scleroderma Renal Crisis: A Multi-Center Study
Arifa Javed¹, Lilit Grigoryan², Alex Luta³, Sumbal Wajid², Lee Shapiro⁴, Virginia Steen⁵ and Swati Mehta⁶, ¹Albany Medical Center, Rensselaer, NY, ²Albany Medical Center, Albany, NY, ³MedStar Georgetown University Hospital, Rockville, MD, ⁴Albany Medical College, Stillwater, NY, ⁵Georgetown University School of Medicine, Washington, DC, ⁶Division of Nephrology, Department of Medicine, Albany Medical Center, Albany, NY
Background/Purpose: Scleroderma renal crisis (SRC) is a rare but life-threatening complication of systemic sclerosis (SSc), characterized by malignant hypertension and acute kidney injury (AKI). Proteinuria when present at the time of…
Abstract Number: 1819 • ACR Convergence 2024
Insulin-like Growth Factor Binding Protein-7 (IGFBP7) Plays a Pathogenic Role in Dermal Fibrosis and Is Increased in Systemic Sclerosis
Ye Jin Jeong¹, Gayathri Guru Murthy¹, Gaochan Wang¹, Tahia Rahman¹ and Benjamin Korman², ¹University of Rochester School of Medicine and Dentistry, Rochester, NY, ²University of Rochester, Rochester, NY
Background/Purpose: Systemic sclerosis (SSc, scleroderma) is an autoimmune connective tissue disease that causes autoimmunity, vascular dysfunction, and fibrosis of the skin, lungs, and other organs.…
Abstract Number: 2052 • ACR Convergence 2024
Role of Scleroderma/myositis-related Autoantibodies Detected by Immunoblot to the Diagnosis of Systemic Autoimmune Rheumatic Diseases in 410 Patients from a Single Referral Center
Carmen Secada Gómez¹, Diana Prieto-Peña², Mónica Renuncio-García³, Juan Irure-Ventura⁴, Marcos Lopez-Hoyos⁵ and Ricardo Blanco-Alonso⁶, ¹Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain, ²Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Cantabria, Spain, ³Division of Immunology, Hospital Universitario Marqués de Valdecilla, Immunopathology Group, IDIVAL, Santander, Spain, ⁴Division of Immunology, Hospital Universitario Marqués de Valdecilla. Immunopathology group, IDIVAL, Santander, Cantabria, Spain, ⁵Division of Immunology, Hospital Universitario Marqués de Valdecilla. Immunopathology group, IDIVAL, Santander, Spain, ⁶Division of Rheumatology, Hospital Universitario Marqués de Valdecilla. IDIVAL, Immunopathology group, Santander, Spain
Background/Purpose: In clinical practice, immunoblot assays are being used more frequently as a diagnostic tool for systemic autoimmune rheumatic diseases (SARDs).Our aim was to evaluate the…
Abstract Number: 2644 • ACR Convergence 2024
Baseline Fibroblast Immunophenotype Predicts Clinical Improvement Among Individuals with Early Diffuse Cutaneous Systemic Sclerosis
Kimberly Lakin¹, Robert Spiera², John Spivack¹, Yaxia Zhang¹, Jessica Gordon³ and dana Orange⁴, ¹Hospital for Special Surgery, New York, NY, ²Scleroderma, Vasculitis, and Myositis Center, Hospital for Special Surgery, Weill Cornell Medical College, New York, NY, ³Division of Rheumatology, Weill Cornell Medical College, New York, NY, ⁴Rockefeller University, New York, NY
Background/Purpose: We have previously shown that fibroblast CD34 is low and alpha-smooth muscle actin (aSMA) is high in severe diffuse cutaneous systemic sclerosis (dcSSc) skin,…
Abstract Number: 0689 • ACR Convergence 2024
Arrhythmic Burden, Myocardial Markers, and Long-term Survival in Distinct Cardiac Magnetic Resonance Subsets of Systemic Sclerosis
Silvia Laura Bosello¹, Enrico De Lorenzis², Giacomo De Luca³, Antonio Tonutti⁴, veronica Batani⁵, Pier Giacomo Cerasuolo⁶, Gerlando Natalello⁷, Lucia Lanzo⁶, Gabriella Alonzi⁶, Silvia Fiore⁶, Stefano Di Murro⁶, Andrea Zoli⁶, Valentina Boni⁶, Riccardo Marano⁸, Francesca Augusta Gabrielli⁹, Francesco Del Galdo¹⁰, Dan Knight¹¹, Vivek Muthurangu¹¹, Christopher Denton¹², Maria De Santis⁴, Marco Matucci Cerinic⁵ and Maria Antonietta D'Agostino¹³, ¹Unit of Rheumatology, Catholic University of the Sacred Heart, Rome, Italy, ²Catholic University of the Sacred Heart, Roma, Rome, Italy, ³Vita-Salute San Raffaele University & IRCCS San Raffaele Hospital, Milan, Milan, Italy, ⁴Rheumatology and Clinical Immunology, IRCCS Humanitas Research Hospital, Rozzano, Italy, ⁵Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy, ⁶Division of Rheumatology, Catholic University of the Sacred Heart, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ⁷Division of Rheumatology - Catholic University of the Sacred Heart, Fondazione Policlinico Universitario Agostino Gemelli, Rome, Rome, Italy, ⁸Department of Diagnostic Imaging, Oncological Radiotherapy and Hematology, Diagnostic Imaging Area, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Rome, Italy, ⁹Department of Cardiovascular and Thoracic Sciences, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy, ¹⁰University of Leeds, Leeds, United Kingdom, ¹¹UCL Department of Cardiac MRI, University College London (Royal Free Campus), London, United Kingdom, ¹²University College London, Northwood, United Kingdom, ¹³Division of Rheumatology, Fondazione Policlinico Universitario Agostino Gemelli IRCSS, Università Cattolica del Sacro Cuore, Rome, Italy
Background/Purpose: Cardiac involvement in Systemic Sclerosis (SSc) is widely recognized as heterogeneous and, when clinically evident, it is associated with a poor prognosis. Recently, five…
Abstract Number: 0954 • ACR Convergence 2024
Increased Proliferating Natural Killer Cells Exhibit an Aberrant Pro-Inflammatory Gene Signature in Systemic Sclerosis Patients
Pietro Bearzi¹, Elena Pachera², Sophie Wagner³, Astrid Hofman⁴, Lumeng Li⁴, Laura Much⁴, Mike Becker⁵, Kristina Bürki⁴, Luca Navarini⁶, Anna-Maria Hoffmann-Vold⁷, Roberto Giacomelli⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Rome, Italy, ⁷Oslo University Hospital, Oslo, Norway, ⁸Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Roma, Italy, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Recent transcriptomic data suggest a prominent involvement of innate immune cells in the pathogenesis of SSc. In this regard, contrasting data on NK cells…
Abstract Number: 1570 • ACR Convergence 2024
Deconvolution of the Lipidomic Signature of Very Early Diagnosis of Systemic Sclerosis (VEDOSS) and Established Disease: Lipid Biomarker Features That Predict Disease Progression in Skin and Lung
Sunhwa Kim¹, Yingtao Bi², Vishal Kakkar³, Rebecca Ross⁴ and Francesco Del Galdo³, ¹AbbVie, South San Francisco, CA, ²Abbvie, Worcester, MA, ³University of Leeds, Leeds, United Kingdom, ⁴Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Systemic sclerosis (SSc) is a heterogeneous autoimmune disease with variable clinical presentations and progression rates. Accurate patient stratification is crucial for optimizing treatment strategies.…

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