Abstract Number: 116 • 2018 ACR/ARHP Annual Meeting
Identifying Matricellular Protein CYR61 As a Potential Anti-Fibrotic and Pro-Angiogenic Mediator in Scleroderma
Background/Purpose: Vascular abnormalities represent a fundamental event in the pathogenesis of scleroderma (SSc) in that endothelial cell (EC) damage triggers a self-fueling process ending in…Abstract Number: 131 • 2018 ACR/ARHP Annual Meeting
Myofibroblast Cells Expression of CD248 May Contribute to Exacerbate Microvascular Damage during Systemic Sclerosis
Background/Purpose: Microvascular rarefaction and tissue fibrosis are the hallmarks of Systemic Sclerosis (SSc). CD248, also known as endosialin, is a transmembrane glycoprotein expressed on key…Abstract Number: 1717 • 2018 ACR/ARHP Annual Meeting
Expert Consensus on the Screening, Treatment, and Management of Patients with Systemic Sclerosis-Interstitial Lung Disease, and the Potential Role of Anti-Fibrotics in a Treatment Paradigm for Systemic Sclerosis-Interstitial Lung Disease: A Delphi Consensus Study
Background/Purpose: Systemic sclerosis (SSc), or scleroderma, is a rare, autoimmune, multisystem connective tissue disorder characterized by vascular damage, autoimmunity, and fibrosis of the skin and…Abstract Number: 767 • 2017 ACR/ARHP Annual Meeting
Elevated MeCP2 Expression in Diffuse Cutaneous Systemic Sclerosis Dermal Fibroblasts Is Associated with Anti-Fibrotic Effects
Background/Purpose: Systemic Sclerosis (SSc) is a multisystem autoimmune connective tissue disorder characterized by vascular injury and fibrosis of the skin and internal organs. Methyl-CpG-binding protein…Abstract Number: 776 • 2017 ACR/ARHP Annual Meeting
Circulating Fibrocytes in Systemic Sclerosis Patients and Healthy Subjects: An in Vitro Study
Background/Purpose: Circulating fibrocytes seem to exert immunomodulatory effects, expressing class II major histocompatability complex molecules (HLA-DP, -DQ, and -DR) and could have a role in…Abstract Number: 806 • 2016 ACR/ARHP Annual Meeting
Single Cell Rnaseq Defines a Unique Transcriptome Profile for Myofibroblasts in the Skin of Patients with Systemic Sclerosis
Background/Purpose: Fibrosis in systemic sclerosis (SSc), as well as a wide variety of other fibrotic diseases, is largely driven by myofibroblasts. Myofibroblasts are known to…Abstract Number: 1855 • 2016 ACR/ARHP Annual Meeting
Phosphodiesterase-5 Inhibitors Attenuate Fibrotic Phenotype and Restore Anti-Fibrotic Resopnses of Cutaneous Fibroblasts in Patients with Scleroderma
Background/Purpose: Scleroderma (SSc) is a chronic autoimmune disease, characterized by excessive fibrosis of skin and internal organs due to uncontrolled proliferation of fibroblasts and deposition…Abstract Number: 1856 • 2016 ACR/ARHP Annual Meeting
Discovery of a Small Molecule Inhibitor of the Wnt Pathway (SM04755) As a Potential Topical Treatment for Scleroderma
Background/Purpose: Scleroderma is an autoimmune fibrotic disease, which presents skin manifestations among others. The Wnt pathway plays an important role in inflammation, skin fibrosis, and…Abstract Number: 3264 • 2015 ACR/ARHP Annual Meeting
Multiplex Serum Protein Analysis in Systemic Sclerosis Defines Potential Anti-Fibrotic Mechanisms and Markers of Response to Hyperimmune Caprine Serum
Background/Purpose: Hyperimmune caprine serum (HICS) administered by subcutaneous injection over 26 weeks has shown benefit for modified Rodnan skin score in diffuse systemic sclerosis…Abstract Number: 1796 • 2014 ACR/ARHP Annual Meeting
Autotaxin Is Highly Expressed in Systemic Sclerosis (SSc) Skin, Mediates Dermal Fibrosis Via IL-6, and Is a Target for SSc Therapy
Background/Purpose Autotaxin (ATX) is an enzyme present in biological fluids that is responsible for the production of the lipid mediator, lysophosphatidic acid (LPA). We previously…Abstract Number: 1725 • 2014 ACR/ARHP Annual Meeting
The SYK Inhibitor Fostamatinib Limits Tissue Damage and Fibrosis in a Bleomycin-Induced Scleroderma MOUSE MODEL
Background/Purpose The possible antifibrotic effects of various kinase inhibitors has been studied before in SSc. Spleen tyrosine kinase (Syk) is a protein tyrosine kinase which…Abstract Number: 969 • 2014 ACR/ARHP Annual Meeting
Caspase-8 Prevents Lung Fibrosis in a Murine SSc-like Disease Model By Preventing Macrophage Differentiation
Background/Purpose Pulmonary fibrosis has emerged as the leading cause of death in patients with Systemic Sclerosis (SSc). Currently available therapies are only marginally effective in…Abstract Number: 964 • 2014 ACR/ARHP Annual Meeting
Skin Collagen Synthesis Rates Distinguish Between Early and Late Diffuse Scleroderma Patients
Background/Purpose The synthesis and degradation of extracellular matrix (ECM), particularly collagen, is one of the central mechanisms perturbed in scleroderma (SSc). Understanding the kinetics of…Abstract Number: 771 • 2014 ACR/ARHP Annual Meeting
Development of a Bifluorescent Lineage Tracker Reporter Mouse Strain to Analyze the Phenotypic Conversion of Endothelial Cells into Myofibroblasts in Vivo. Application to Study the Synergistic Effects of Endothelin-1 on TGF-β1-Induced Endothelial-to-Mesenchymal Transition
Background/Purpose: Endothelial-to-mesenchymal transition (EndoMT) may be a crucial pathway in generating activated myofibroblasts, cells that play a pivotal role in the development of tissue and…