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: 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…
Abstract Number: 0784 • ACR Convergence 2024
RUNX1 Is Expressed in a Subpopulation of Dermal Fibroblasts and Is Increased with Systemic Sclerosis Disease Severity
Rezvan Parvizi¹, Zhiyun Gong², Helen Jarnagin², Tamar Abel², Dillon Popovich³, Madeline Morrisson⁴, Tammara Wood⁵, Sasha Shenk⁶, Monique Hinchcliff⁷, jonathan Garlick⁶, Patricia Pioli⁸ and Michael Whitfield¹, ¹Geisel School of Medicine at Dartmouth, Hanover, NH, ²Dartmouth College, Lebanon, NH, ³Dartmouth College, West Lebanon, NH, ⁴Geisel School of Medicine at Dartmouth College, Hanover, NH, ⁵Dartmouth, Hanover, NH, ⁶Tufts University, Boston, MA, ⁷Yale School of Medicine, Westport, CT, ⁸Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: Systemic Sclerosis (SSc) skin fibrosis results in complex changes in transcriptional and signaling pathways in the skin. Through transcription factor activity network analyses, the…
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: 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: 2462 • ACR Convergence 2024
A Potent Inhibitor of PAI-1, MDI-2517, Mitigates Disease Severity in Preclinical Models of Systemic Sclerosis
Enming Su¹, Pei-Suen Tsou¹, Mark Warnock², Natalya Subbotina¹, Kris Mann¹, Sirapa Vichaikul¹, Xianying Xing¹, Enze Xing¹, Olesya Plazyo¹, Rachael Wasikowski¹, Lam C. Tsoi³, Mark Weinberg⁴, Cory D. Emal⁵, Dinesh Khanna¹, John Varga¹, Thomas H. Sisson¹, Johann Gudjonsson¹ and Daniel Lawrence², ¹University of Michigan, Ann Arbor, MI, ²Division of Cardiovascular Medicine, University of Michigan, Ann Arbor, MI, ³Michigan, Dept. of Dermatology, Ann Arbor, MI, ⁴MDI, Ann Arbor, MI, ⁵EMU, Ann Arbor, MI
Background/Purpose: Systemic sclerosis (SSc) is a complex and heterogeneous condition characterized by progressive fibrosis in multiple organs. Currently, there is no known cure for SSc,…
Abstract Number: 0930 • ACR Convergence 2024
The Common Form of the Inflammatory Skin Disease Hidradenitis Suppurativa Is Associated with Low Nicastrin Expression in Dermal Fibroblasts
Kaitlin Williams, Beita Badiei, Hana Minsky and Luis Garza, Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: Hidradenitis suppurativa (HS) is a chronic inflammatory skin condition characterized by painful nodules and abscesses in intertriginous areas. Despite its increasing prevalence, the etiology…
Abstract Number: 2473 • ACR Convergence 2024
Criterion Validity of Modified Rodnan Skin Score: Does It Capture Skin Thickness or Hardening? Results of a Large Skin Histology Study
Shervin Assassi¹, Ruhani Desai², Jeffrey Browning³, Samuel Theodore¹, Meng Zhang¹, Brian Skaug¹, Jerry Alonso¹, Zsuzsanna McMahan¹, Maureen Mayes¹ and Minghua Wu¹, ¹UTHealth Houston Division of Rheumatology, Houston, TX, ²UTHealth Houston Division of Rheumatology, DeLand, FL, ³Boston University, Cambridge, MA
Background/Purpose: Modified Rodnan Skin Score is a widely used outcome measure for skin involvement in systemic sclerosis (SSc). Its criterion validity (comparison to the gold…
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: 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: 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…

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