Abstracts tagged "Fibroblasts, Dermal"

Abstract Number: L18 • ACR Convergence 2024
CCL19+ Fibroblasts Orchestrate Fibrotic Microenvironment via CCL19-CCR7 Axis in Systemic Sclerosis
Wei Guo¹, Zhaohua Li², Dan Xu² and Rong Mu², ¹Department of Rheumatology and Immunology, Peking University Third Hospital, Beijing, China, ²Peking University Third Hospital, Beijing, China
Background/Purpose: Understanding the roles of diverse fibroblast subsets is of great importance in elucidating the pathogenesis of fibrosis in systemic sclerosis (SSc). However, how the…
Abstract Number: 1643 • ACR Convergence 2024
Single Nuclei Multiome and Spatial Transcriptomic Analysis of Early, Untreated SSc Skin Identifies Signaling Interactions Between Macrophages and Fibroblasts
Helen Jarnagin¹, Dillon Popovich², Rezvan Parvizi³, Rosemary Gedert⁴, Lam C. Tsoi⁵, Rachael Wasikowski⁵, Zhiyun Gong¹, Madeline Morrisson⁶, Laurent Perreard⁷, Fred Kolling IV⁷, Dinesh Khanna⁴, Johann Gudjonsson⁴ and Michael Whitfield³, ¹Dartmouth College, Lebanon, NH, ²Dartmouth College, West Lebanon, NH, ³Geisel School of Medicine at Dartmouth, Hanover, NH, ⁴University of Michigan, Ann Arbor, MI, ⁵Michigan, Dept. of Dermatology, Ann Arbor, MI, ⁶Geisel School of Medicine at Dartmouth College, Hanover, NH, ⁷Geisel School of Medicine, Dartmouth College, Lebanon
Background/Purpose: We generated a vertically integrated dataset on treatment naïve patients with dcSSc (diffuse Systemic Sclerosis) skin that includes bulk RNA-seq, single nuclei multiome, and…
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: 1770 • ACR Convergence 2024
Assessing S100A4 and Myofibroblast Phenotypes in the Pathogenesis of Cardiac and Cutaneous Neonatal Lupus
Nalani Sachan¹, Christina Firl², Philip Carlucci³, Nicola Fraser², Robert Clancy⁴ and Jill Buyon⁵, ¹NYU Grossman School of Medicine, New York, NY, ²NYU Langone Health, New York, NY, ³New York University School of Medicine, New York, NY, ⁴Columbia University Medical Center, New York, NY, ⁵New York University Grossman School of Medicine, New York, NY
Background/Purpose: Neonatal lupus (NL), driven by fetal exposure to maternal anti-SSA/Ro autoantibodies, typically results in permanent cardiac manifestations in utero and/or a transient rash postnatally.…
Abstract Number: 1815 • ACR Convergence 2024
Leveraging Novel Systemic Sclerosis Disease Signatures to Build a Humanized Drug Discovery Funnel
Lauren Reinke-Breen¹, Laura Leys¹, Sunhwa Kim², Lauren Olson¹, Anastasia Marinopoulos¹, Chris Butler¹, Jennifer Van Camp¹ and Lisa Hazelwood¹, ¹AbbVie, North Chicago, IL, ²AbbVie, South San Francisco, CA
Background/Purpose: Systemic sclerosis (SSc) is a chronic, progressive autoimmune disorder with significant morbidity and mortality. The lack of effective therapies for SSc is, in part,…
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: 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: 1820 • ACR Convergence 2024
Disruption of Ca2+ Homeostasis and Enhanced Ca2+ Signaling in Scleroderma Fibroblasts Is Due to the Vicious Cycle Formed by Mitochondrial-derived Reactive Oxygen Species, CaMKII, and ER Extrusion Pump RYR2
Yuzo Ikari, William Brodie, Megan Mattichak, Sirapa Vichaikul, Qi Wu, Dinesh Khanna and Eliza Pei-Suen Tsou, University of Michigan, Ann Arbor, MI
Background/Purpose: Prior research has suggested that manipulating intracellular Ca2+ concentrations might yield anti-fibrotic benefits across various experimental models. Moreover, inhibiting downstream calcium-binding proteins, such as…
Abstract Number: 1821 • ACR Convergence 2024
Single-Cell RNA SequencingBioinformatic Pipeline Optimized for Non-Coding GenesIdentified LINC01503 and MIR193-BHG as Potential Pathogenic Effectors in Systemic Sclerosis Fibroblasts
Sophie Wagner¹, Elena Pachera², Gino Andrea Bonazza³, Celina Geiss⁴, Astrid Hofman⁴, Laura Much⁴, Lumeng Li⁴, Pietro Bearzi³, Anna-Maria Hoffmann-Vold⁵ and Oliver Distler⁶, ¹University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Oslo University Hospital, Oslo, Norway, ⁶Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Long non-coding RNAs (lncRNA) are a class of transcripts that regulate gene expression. Currently, only few lncRNAs have been linked to SSc pathogenesis. As…
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: 1827 • ACR Convergence 2024
Characterization of Fibroblast Subpopulations and Their Cellular Local Environments in Systemic Sclerosis Using Imaging Mass Cytometry
Aleix Rigau¹, Minrui Liang², Veda Devakumar², Ranjana Neelagar², Alexandru-Emil Matei², Andrea-Hermina Györfi², Christina Bergmann³, Tim Filla², Vladyslav Fedorchenko¹, Georg Schett⁴, Jörg Distler⁵ and Yi-Nan Li², ¹Friedrich-Alexander Universität Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ²Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Düsseldorf, Germany, ³Department Internal Medicine III, Friedrich-Alexander-University (FAU) Erlangen-Nurnber, Frankfurt, Germany, ⁴Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany, ⁵Universitätsklinikum Düsseldorf and Heinrich-Heine-Universität Düsseldorf, Duesseldorf, Germany
Background/Purpose: Single-cell transcriptomic data has shown that fibroblasts exhibit heterogeneity, with subpopulations that have diverse functional characteristics. However, the conventional single-cell methods are unable to…
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…
Abstract Number: 1831 • ACR Convergence 2024
Role of Long Non-Coding RNA H19 in Mediating Skin Fibrosis in Systemic Sclerosis
Begoña Caballero-Ruiz¹, Christopher Wasson², Rebecca Ross³ and Francesco Del Galdo², ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ²University of Leeds, Leeds, United Kingdom, ³Medicine and Health, University of Leeds, Leeds, United Kingdom
Background/Purpose: Systemic Sclerosis (SSc) is an immune driven condition leading to skin fibrosis, as well as internal organs. Dermal fibroblasts are the key cellular elements…
Abstract Number: 0089 • ACR Convergence 2024
Single-Cell RNA Sequencing Outlines the Mechanisms of Vgll3-Driven Myofibroblasts Differentiation in Promoting Fibrosis in Cutaneous Lupus
Mehrnaz Gharaee-Kermani, Allison Billi, Poulami Dey, Jacob Martens, Marisa Hildebrandt, J. Michelle Kahlenberg and Johann Gudjonsson, University of Michigan, Ann Arbor, MI
Background/Purpose: Skin fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling. Some subtypes of cutaneous lupus erythematosus (CLE), particularly discoid lupus erythematosus…
Abstract Number: 1834 • ACR Convergence 2024
Altered Mechanotransduction via Myosin II Contributes to Collagen and IL-6 Production in Systemic Sclerosis Skin
Barbara Russo¹, Shutova Maria², Fanny Noulet², Geroge-Radu Romanescu³, Nicolo brembilla² and Wolf-Henning Boehncke², ¹Hopitaux Universitaire de Geneva, Geneva, Geneve, Switzerland, ²Hôpitaux Universitaires des Genève, Geneva, Geneve, Switzerland, ³University of Geneva, Geneva, Geneve, Switzerland
Background/Purpose: Scleroderma (SSc), an autoimmune disease, features progressive fibrosis, leading to significant morbidity and mortality. Current therapies manage symptoms but lack efficacy in directly targeting…

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