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.…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
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
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
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: 0892 • ACR Convergence 2023
Single-Cell RNA Sequencing Reveals Keratinocytes and Fibroblasts Drive Hippo and TGFb Dysregulation and Fibrosis in Epidermal Overexpression of VGLL3 in Cutaneous Lupus
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast (MYOFB) accumulation, and extracellular matrix remodeling. In some subtypes of cutaneous lupus erythematosus (CLE), particularly discoid lupus…Abstract Number: 1663 • ACR Convergence 2023
Clinically Severe Systemic Sclerosis Skin Harbors Inflammatory Fibroblasts Associated with Lymphocytes and Plasmacytoid Dendritic Cells
Background/Purpose: We have previously shown that skin fibroblast activation is related to clinical severity and resolves with improvement in diffuse systemic sclerosis (dcSSc). The immune…Abstract Number: 0934 • ACR Convergence 2023
Anti-fibrotic Effects of MT-5562, a Novel Potent Selective Autotaxin Inhibitor, in Preclinical Studies: Roles of Lysophosphatidic Acid in Autoimmune Diseases and Clues to Treat Skin and Lung Fibrosis in Systemic Sclerosis
Background/Purpose: Targeted small-molecule therapies to autotaxin (ATX), the lysophosphatidic acid (LPA)-producing enzyme, have been shown to be potentially effective in systemic sclerosis (SSc), but the…Abstract Number: 1664 • ACR Convergence 2023
Fibroblast Specific Interferon Regulatory Factor 7 (IRF7) Expression Is a Key Link Between Type I Interferon Activation and the Exaggerated Dermal and Pulmonary Fibrosis in Systemic Sclerosis
Background/Purpose: A prominent interferon (IFN) activation signature has been observed at the peripheral blood and end-organ levels in systemic sclerosis (SSc). However, the mechanisms by…Abstract Number: 0936 • ACR Convergence 2023
Impaired DNA Repair Responses Activate a Novel FOXO1-dependent Metabolic Remodelling in Patients with Progressive Systemic Sclerosis to Promote Fibrosis
Background/Purpose: Systemic sclerosis (SSc) is a deadly disease characterized by immune dysregulation, vasculopathy, and fibrosis. SSc is the most lethal rheumatic disease associated with the…Abstract Number: 2367 • ACR Convergence 2023
Characterization of the SFRP4+ PRG4+ Fibroblast Subpopulation in the Dermis of Healthy Skin and Its Relationship to SFRP4+ PRG4- Myofibroblasts in the Skin of Patients with Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a debilitating autoimmune disease characterized by extensive fibrosis affecting multiple organs. The fibrosis arises from the excessive production of collagen…Abstract Number: 0941 • ACR Convergence 2023
RUNX1 Expression and Binding Site Accessibility Is Associated with Increased Disease Severity in Systemic Sclerosis
Background/Purpose: Runt-related transcription factor 1 (RUNX1) is a member of the core-binding factor family that regulates proliferation, differentiation, and cell survival in multiple cell lineages.…
ACR Meeting Abstracts