Abstract Number: L18 • ACR Convergence 2024
CCL19+ Fibroblasts Orchestrate Fibrotic Microenvironment via CCL19-CCR7 Axis in Systemic Sclerosis
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: 0784 • ACR Convergence 2024
RUNX1 Is Expressed in a Subpopulation of Dermal Fibroblasts and Is Increased with Systemic Sclerosis Disease Severity
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
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
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
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
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
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
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
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
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
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
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
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
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
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.…
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