Abstract Number: 1168 • ACR Convergence 2022
Dermal Fibroblast-derived Exosomes Drive Profibrotic Macrophage Activation in Systemic Sclerosis
Background/Purpose: Macrophage (MØ) activation derives from coordination of signals received in local tissue microenvironments. In prior studies, we demonstrated that cocultured MØs and fibroblasts from…Abstract Number: 1170 • ACR Convergence 2022
Systemic Sclerosis Dermal Fibroblast-derived Exosomes Trigger a Type 1 Interferon Rresponse in Keratinocytes Through TBK1
Background/Purpose: Elevated Type I IFN response is present in blood and affected tissues in systemic sclerosis (SSc) and correlates with disease activity and response to…Abstract Number: 1172 • ACR Convergence 2022
Transcription Analysis of Macrophages Reveals Important Changes Induced by Systemic Sclerosis Fibroblasts
Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune connective tissue disease characterized by immune system activation, endothelial dysfunction and widespread tissue fibrosis. Activation of the monocyte/macrophage…Abstract Number: 1176 • ACR Convergence 2022
Immunoglobulins G from Systemic Sclerosis Patients May Alter the Secretome of Dermal Fibroblasts
Background/Purpose: Autoantibodies (Aab) are frequent in systemic sclerosis (SSc).Recently, it has been shown that immunoglobulins G (IgG) from SSc promoted a proinflammatory and profibrotic phenotype…Abstract Number: 1181 • ACR Convergence 2022
Hippo Pathway Effectors Promote and Maintain Myofibroblast Differentiation and Endothelial-to-mesenchymal Transition in Systemic Sclerosis
Background/Purpose: Systemic sclerosis (SSc) is a devastating autoimmune disease characterized by excessive production and accumulation of extracellular matrix molecules leading to fibrosis of skin and…Abstract Number: 1183 • ACR Convergence 2022
Functionally Distinct Fibroblast Populations in Systemic Sclerosis Skin Revealed by Explant Tissue Characterization and Single-cell RNAseq
Background/Purpose: Traditional studies of skin fibroblasts in SSc focus on the early "migratory" fibroblast population derived from explant culture of skin biopsies. Single-cell RNAseq has…Abstract Number: 1184 • ACR Convergence 2022
Intracellular Calcium Signals Regulate the Pro-fibrotic Phenotype of Scleroderma Fibroblasts via Calcium/calmodulin-dependent Kinase II
Background/Purpose: We performed an in-depth analysis of the involvement of histone reader bromodomain extra-terminal proteins (BETs) in scleroderma (SSc) fibrosis and showed that JQ1, a…Abstract Number: 1185 • ACR Convergence 2022
Interleukin-11 Promotes Fibrosis Through Classic and Trans-signaling Pathway in Systemic Sclerosis
Background/Purpose: Interleukin-11 (IL-11) was found significantly upregulated in Systemic sclerosis (SSc), the aim of this study is to explore the pathological role of IL-11 and…Abstract Number: 1624 • ACR Convergence 2022
Blocking IL-1, IL-33 and IL-36 Signaling with the Anti-IL1RAP Antibody mCAN10 Ameliorates Inflammation and Fibrosis in Preclinical Models of Systemic Sclerosis
Background/Purpose: The IL-1 receptor accessory protein (IL1RAP) is a co-receptor required for signaling through the IL-1, IL-33, and IL-36 receptors. IL1RAP-dependent signaling has been implicated…Abstract Number: 0645 • ACR Convergence 2022
Lupus Fibroblasts from Non-lesional Skin Exhibit Exaggerated Responses to Inflammatory Cytokines and Upregulate Pro-fibrotic Collagens in Patients with Scarring Lesions
Background/Purpose: Cutaneous lupus erythematosus (CLE) is a manifestation of systemic lupus erythematosus (SLE) that can cause significant patient distress and disfiguration secondary to scar. Scarring…Abstract Number: 0661 • ACR Convergence 2022
Cutaneous Type I IFN Responses in Systemic Lupus Erythematosus Are Associated with Inflammatory Phenotype and Altered Wound Healing Function of Lupus Fibroblasts from Non-Lesional Skin
Background/Purpose: Cutaneous lupus erythematosus (CLE) is a heterogenous, disfiguring, and difficult-to-treat manifestation of systemic lupus erythematosus (SLE) with scar formation in CLE subtypes such as…Abstract Number: 1065 • ACR Convergence 2022
Biosamples from VEDOSS Patients Show Pathological Signs of SSc: Opportunity for a Biololgical Diagnosis of Disease
Background/Purpose: The VEDOSS study has recently indicated that more than 50% of patients affected by Raynaud’s phenomenon (RP) and specific SSc anti-nuclear antibodies (ANA) and/or…Abstract Number: 0542 • ACR Convergence 2021
Functional Characterization of Glycoprotein Nonmetastatic Melanoma Protein B in Scleroderma Fibrosis
Background/Purpose: Glycoprotein nonmetastatic melanoma protein B (GPNMB) is widely expressed on stromal cells and immune cells. It is involved in various cell functions such as…Abstract Number: 0544 • ACR Convergence 2021
Targeting CD13/aminopeptidase N as a Novel Therapeutic Approach for Scleroderma Fibrosis
Background/Purpose: Aminopeptidase N, also known as CD13, is a Zn2+-dependent membrane bound ectopeptidase widely expressed in mammalian cells including rheumatoid arthritis (RA) fibroblast-like synoviocytes (FLS),…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…
ACR Meeting Abstracts