Abstract Number: 0952 • ACR Convergence 2024
Single Cell RNA-seq of Scleroderma-associated Interstitial Lung Disease Lung Explants Reveals an Active, Cytotoxic Natural Killer Cell Population
Background/Purpose: Scleroderma-associated interstitial lung disease (SSc-ILD) is the leading cause of death among SSc patients. Loss of alveolar epithelial cells (AEC) and bronchial cell hyperplasia,…Abstract Number: 2540 • ACR Convergence 2024
Characterizing the Functional Role of Type I Interferons in Inflammatory Arthritis
Background/Purpose: Rheumatoid arthritis (RA) is a chronic autoimmune disease characterized by synovial inflammation due to cellular infiltration and inflammatory mediators. Type I interferons (IFN) have…Abstract Number: 0964 • ACR Convergence 2024
Engaging the PD-1 Pathway Attenuates Inflammation Associated Fibrosis in Systemic Sclerosis Fibroblasts and a Preclinical Mouse Model
Background/Purpose: The precise molecular mechanisms driving fibrosis in diffuse cutaneous systemic sclerosis (dcSSc) remain to be elucidated. The immune regulatory programmed cell death protein 1…Abstract Number: 2542 • ACR Convergence 2024
Group 2 Innate Lymphoid Cells Aggravates Development of Inflammatory Arthritis
Background/Purpose: Activation of fibroblast-like synoviocytes (FLS) by immune cells drives inflammation and joint damage in rheumatoid arthritis (RA). Hence, understanding factors that induce destructive behaviors…Abstract Number: 0967 • ACR Convergence 2024
Transcriptional Heterogeneity of Immune Cells in the Esophagus of Systemic Sclerosis Patients: A Comparison of Upper and Lower Esophageal Regions
Background/Purpose: Systemic sclerosis (SSc) is characterized by an initial inflammatory phase followed by fibrosis. Esophageal dysfunction in SSc is associated with gastroesophageal reflux, leading to…Abstract Number: 1701 • ACR Convergence 2024
STING Pathway Activity in SLE Patient Serum Correlates with NFkB Activation, Autoantibody Levels, and a Unique Cytokine Profile That Drives Disease Activity
Background/Purpose: Systemic lupus erythematosus (SLE) is a heterogenous systemic autoimmune disease which is associated with innate immune activation, type I IFN, inflammasome and NFkB-related cytokines.…Abstract Number: 1712 • ACR Convergence 2024
Transcriptome Analysis Characterizes the Role of Monocytes in Ankylosing Spondylitis with TNF-blocker Treatment
Background/Purpose: Ankylosing spondylitis (AS) is an autoimmune and auto-inflammatory disease characterized by chronic inflammation of the spine, sacroiliac joint, and occasionally peripheral joints. TNF-α inhibitor…Abstract Number: 1766 • ACR Convergence 2024
STAT2-Associated Type I Interferonopathy: A Masquerade of Infectious Susceptibility
Background/Purpose: Type I IFN (IFN-I) signaling is a potent inflammatory pathway fundamental to antiviral immunity. In humans, loss of IFN-I activity underlies severe viral disease,…Abstract Number: L02 • ACR Convergence 2023
Mutational Analysis of UNC93B1 Identifies Regulatory Regions Mutated in Human SLE
Background/Purpose: Endosomal nucleic acid-sensing toll-like receptors 3, 7 and 9 are key innate immune sensors of dsRNA, ssRNA and ssDNA, respectively, whose activities must be…Abstract Number: L08 • ACR Convergence 2023
Early Clinical Development of CIT-013, a First in Class NETosis Inhibitor, in a Randomized Phase I Dose Escalation Study in Healthy Volunteers Demonstrating Potent Inhibition of LPS Induced Neutrophil Extracellular Trap Formation
Background/Purpose: Aberrant Neutrophil Extracellular Traps (NETs) production contributes to the pathophysiology of multiple inflammatory and autoimmune diseases such as Rheumatoid arthritis (RA) Hidradenitis suppurativa (HS),…Abstract Number: 0920 • ACR Convergence 2023
Proinflammatory Neutrophils and NETs Mediate Skin and Kidney Inflammation During Lupus Flare in Asymptomatic Lupus-prone Mice Triggered by UVB
Background/Purpose: Ultraviolet B (UVB) exposure triggers lupus flare by worsening both skin lesions and systemic symptoms, i.e. lupus nephritis. We recently reported that UVB exposure…Abstract Number: 0040 • ACR Convergence 2023
Non-canonical NF-κB Signalling Is Required for Extrathymic AIRE Expression and Immunoregulatory Molecules in Cells of the Dendritic Lineage
Background/Purpose: The transcription factor Autoimmune Regulator (AIRE) is crucial for the establishment of central tolerance in the thymus. Recently, peripheral CD45+ extrathymic AIRE-expressing cells (eTACs)…Abstract Number: 0940 • ACR Convergence 2023
A Novel Therapeutic Opportunity in Systemic Sclerosis: The Fibrolytic Activities of a Specialized Macrophage Secretome
Background/Purpose: Systemic sclerosis (SSc) a complex and rare immune-mediated connective tissue disorder characterized by microvascular damage, inflammatory cell infiltration, and excessive deposition of extracellular matrix…Abstract Number: 0041 • ACR Convergence 2023
Interactions Between Synovial Fibroblasts and Macrophages: Implications for Tissue Remodeling in Chronic Inflammatory Diseases and Macrophage Differentiation in Response to the Immune Microenvironment
Background/Purpose: Activated macrophages (Mph) can be subdivided in at least 2 major subgroups according to their polarization into classical pro-(M1-Mph) or anti-inflammatory (M2-Mph) subtypes. Together…Abstract Number: 0947 • ACR Convergence 2023
Fcγ Receptors Define Pro-Phagocytic Macrophages and Trigger Pro-Inflammatory Responses in Patients with Systemic Sclerosis
Background/Purpose: Fcy receptors (FcγR) are opsonic phagocytic receptors, requiring tight regulations to prevent uncontrolled activation of pro-inflammatory phagocytosis. SSc macrophages display an alternatively-activated profibrotic phenotype.…
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