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: 0957 • ACR Convergence 2023
Rationale for Targeting Insulin-like Growth Factor Signalling in Systemic Sclerosis
Background/Purpose: Previous studies have shown that insulin like growth factor (IGF) pathways may promote extracellular matrix deposition and be upregulated in systemic sclerosis (SSc). Teprotumumab,…Abstract Number: 0960 • ACR Convergence 2023
MiR-3606-3p Alleviates Skin Fibrosis by Suppressing Fibroblast Inflammation and Migration via Inhibiting GAB1 and ITGAV
Background/Purpose: Systemic sclerosis (SSc) and keloid are typical skin fibrotic diseases with unclear epigenetic mechanisms and clinical targets. As an important epigenetic regulatory factor, microRNAs…Abstract Number: 0085 • ACR Convergence 2023
Fibroblasts Promote Upregulation of Cannabinoid Type 2 Receptor on Inflammatory Cells in Dermatomyositis
Background/Purpose: Dermatomyositis (DM) is a chronic, systemic autoimmune disease affecting the skin, muscle, and lungs. The activation of CB2R has been shown to reduce several,…Abstract Number: 0963 • ACR Convergence 2023
Anti-Topoisomerase Antibody in dcSSc: Unravelling Its Intracellular Effects in Systemic Sclerosis
Background/Purpose: The presence of autoantibodies, especially directed towards topoisomerase I (ATA), in diffuse cutaneous Systemic Sclerosis (dcSSc) is known to be associated with severe clinical…Abstract Number: 0787 • ACR Convergence 2023
Understanding Distinct Resident and Migratory Fibroblast Populations in Systemic Sclerosis Skin Through Single-cell RNAseq and Immunohistochemistry
Background/Purpose: We previously described distinct migratory and resident fibroblast populations from lesional skin biopsies in SSc patients. Both populations are profibrotic compared with those from…Abstract Number: 0964 • ACR Convergence 2023
Systemic Sclerosis (SSc) Dermal Fibroblasts Show Shortened Primary Cilia Due to Aberrant Aurora a Kinase Activation Independently of Transforming Growth Factor β Signalling
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disorder characterised by abnormal activation of tissue fibroblasts, resulting in tissue and vascular fibrosis of the skin and…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…