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…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.…Abstract Number: 0942 • ACR Convergence 2023
G Protein-coupled Receptor Kinase 5 in Fibrotic Tissue Remodeling
Background/Purpose: G-protein coupled receptor kinase 5, GRK5, is a central regulator of G protein-coupled receptors (GPCRs), a vast family of cell surface receptors involved in…Abstract Number: 0943 • ACR Convergence 2023
Attenuation of Fibroblast Activation and Fibrosis by Adropin in a Hedgehog-Dependent Manner
Background/Purpose: Adropin is a secretory protein encoded by the energy homeostasis - associated (ENHO) gene. Emerging evidence indicate its role in metabolism and energy homeostasis,…Abstract Number: 0945 • ACR Convergence 2023
The Nuclear Receptor TR4 Orchestrates Cytoskeletal Organization in a Gα12/ROCK-dependent Manner to Promote Myofibroblast Differentiation and Tissue Fibrosis in Systemic Sclerosis
Background/Purpose: Members of the superfamily of nuclear receptors have been implicated in inflammatory processes and pathologic tissue remodeling and have emerged as attractive targets for…Abstract Number: 0946 • ACR Convergence 2023
Immunoglobulins G Purified from Systemic Sclerosis Patients Influence Cytoplasmic and Nuclear Proteins Profile in Dermal Fibroblasts
Background/Purpose: Autoantibodies (Aab) are frequent in systemic sclerosis (SSc). While recognized as potent biomarkers, their pathogenic role is unclear. Recently, we showed that immunoglobulins G…Abstract Number: 007 • 2023 Pediatric Rheumatology Symposium
Single Cell RNA Sequencing Analysis of the Skin to Evaluate the Effect of Autologous Stem Cell Transplant on Fibroblast Populations in Juvenile Onset Systemic Sclerosis
Background/Purpose: Juvenile systemic sclerosis (JSSc) is a rare autoimmune disease associated with high morbidity. Inflammatory driven multi-organ fibrosis is similar to adult-onset SSc, with 40%…
ACR Meeting Abstracts