Abstract Number: 0551 • ACR Convergence 2021
Metabolic Intermediate Dimethyl-Alpha-Ketoglutarate Is a Novel Repressor of Pathogenic Myofibroblast Reprogramming and Skin Fibrosis in Systemic Sclerosis
Background/Purpose: Metabolic perturbations drive fibroblast-to-myofibroblast reprogramming and tissue fibrosis. Restoring perturbed metabolism might represent a new antifibrotic strategy. Here we explored the capacity of dimethyl-alpha-ketoglutarate…Abstract Number: 1858 • ACR Convergence 2021
Serum Interferon Score Is a Biomarker of Active Disease in Patients with Early Diffuse Cutaneous Systemic Sclerosis Enrolled in the Prospective Registry of Early Systemic Sclerosis (PRESS) Cohort
Background/Purpose: Several published studies have demonstrated activation of the interferon type 1 (IFN) pathway in sera from patients with systemic sclerosis (SSc). Specifically, levels of…Abstract Number: 025 • 2020 Pediatric Rheumatology Symposium
HLA Genetic Signatures Associated with Inflammatory Sub-type in Juvenile Localized Scleroderma
Background/Purpose: Juvenile localized scleroderma (jLS) is an autoimmune disease of the skin and underlying tissue that is characterized by an earlier inflammatory infiltrate, followed by…Abstract Number: 1043 • 2019 ACR/ARP Annual Meeting
The Metabolic Intermediate Alpha-Ketoglutarate Suppresses the TGFβ-driven Profibrotic Responses of Dermal Fibroblasts
Background/Purpose: Metabolic perturbations are emerging as drivers of fibroblast activation in fibrosis. Transcriptomic analyses have shown the enrichment of glycolysis and suppression of tricarboxylic acid…Abstract Number: 1059 • 2019 ACR/ARP Annual Meeting
BDCA2 Targeting of Human Plasmacytoid Dendritic Cells via CBS004 Reverts Dependent IFN Activation and Tissue Fibrosis in vitro and in vivo
Background/Purpose: Human plasmacytoid dendritic cells (pDCs) have been implicated in the pathogenesis of Systemic Sclerosis (SSc) through their ability to infiltrate the skin and secrete…Abstract Number: 1072 • 2019 ACR/ARP Annual Meeting
CCR2+ Circulating Monocytes Contribute to the Survival of ADSC in Bleomycin-Induced Skin Fibrosis
Background/Purpose: Monocytes and monocyte-derived cells play a crucial role during homeostasis and also during the development of various inflammatory diseases including skin fibrosis. Ly6Chi inflammatory…Abstract Number: 720 • 2019 ACR/ARP Annual Meeting
Baseline Subject Demographics and Disease Characteristics in a Phase 3 Study of Safety and Efficacy of Lenabasum in Diffuse Cutaneous Systemic Sclerosis
Background/Purpose: We report the baseline characteristics of a large cohort of diffuse cutaneous systemic sclerosis (dcSSc) patients enrolled in a Phase 3 trial of lenabasum,…Abstract Number: 865 • 2019 ACR/ARP Annual Meeting
Safety and Efficacy of Lenabasum at 21 Months in an Open-Label Extension of a Phase 2 Study in Diffuse Cutaneous Systemic Sclerosis Subjects
Background/Purpose: Lenabasum is a synthetic, non-immunosuppressive, selective cannabinoid receptor type 2 agonist that activates resolution of innate immune responses and limits fibrosis in animal models…Abstract Number: 1042 • 2019 ACR/ARP Annual Meeting
Thy-1 (CD90) as a Novel Marker for Tracking in Vivo Skin Fibrosis
Background/Purpose: Thy-1 (CD90) is a cell surface marker which is found primarily on fibroblasts and whose expression has previously been shown to correlate with pathologic…Abstract Number: 2693 • 2018 ACR/ARHP Annual Meeting
What Is the Effect of Cyclophosphamide Iv Pulse Therapy in Patients with Diffuse Cutaneous Systemic Sclerosis on Skin Involvement: An Observational Study
Background/Purpose: Patients with systemic sclerosis who have proximal skin involvement are classified as diffuse cutaneous systemic sclerosis (DcSSc). Patients with progressive skin involvement have worse prognosis.…Abstract Number: 1714 • 2017 ACR/ARHP Annual Meeting
Inhibition of Hedgehog Acyltransferase Alleviates the Profibrotic Effects of Transforming Growth Factor β in Systemic Sclerosis
Background/Purpose: Hedgehog acyltransferase (Hhat) catalyzes the attachment of the fatty acid palmitate onto Sonic Hedgehog (Shh), a modification essential for Shh signaling activity. Palmitoylation of…Abstract Number: 2885 • 2017 ACR/ARHP Annual Meeting
Tadalafil Reduces Skin Fibrosis and Profibrotic Genes Expression in Patients with Systemic Sclerosis
Background/Purpose: Currently, drugs that modify skin fibrosis in Systemic Sclerosis (SSc) have efficacy in certain subgroups of patients only. Phosphodiesterase-5 inhibitors (PDE5i) are known to…Abstract Number: 2974 • 2015 ACR/ARHP Annual Meeting
Insulin Resistance Is Associated with the Digital Ulcer in Patients with Systemic Sclerosis
Background/Purpose: Growing evidence supports the view that systemic sclerosis (SSc) is a vascular disease mediated by autoimmunity and evolving into progressive tissue fibrosis. Several vascular…Abstract Number: 1697 • 2014 ACR/ARHP Annual Meeting
High Frequency Skin Ultrasound Detects Subclinical Diffuse Dermal Involvement in Patients with Limited Cutaneous Systemic Sclerosis
Background/Purpose . The modified Rodnan’s skin score (mRSS) is the validated method to detect both severity and extension of skin involvement, and high frequency skin…