Abstract Number: 1191 • ACR Convergence 2025
Effect of ER Stress Inhibition With 4-Phenylbutyric Acid on Disease Phenotype in a Mouse Model of Myositis
Background/Purpose: Dysregulation of the ER stress and interferon (IFN) pathways play a major role in the pathophysiology of autoimmune myositis. Upregulation of ER stress markers…Abstract Number: 0802 • ACR Convergence 2025
Remission from cutaneous manifestations of lupus with enpatoran, a first-in-class oral small molecule toll-like receptor 7/8 inhibitor: pooled post-hoc exploratory analysis from a randomized placebo-controlled Phase II study
Background/Purpose: Cutaneous manifestations of lupus are highly prevalent and have a significant impact on patients’ physical and mental health and wellbeing; however, no targeted therapy…Abstract Number: 1826 • ACR Convergence 2025
Single Nuclei Multiome of JDM Muscle Biopsies Reveals Novel Upregulation of Inflammatory and Vascular Pathways
Background/Purpose: Juvenile dermatomyositis (JDM) is a multisystem vasculopathy and inflammatory myopathy characterized by proximal muscle weakness, distinct rash, and risk of long-term complications such as…Abstract Number: 1185 • ACR Convergence 2025
Pulmonary Arterial Hypertension in Adults with Still’s Disease: Another Pulmonary Manifestation Associated with HLA-DRB1*15
Background/Purpose: Inflammatory lung disease (ILD) in Still’s disease (SD) has recently been described. Pulmonary arterial hypertension (PAH), a rare subtype of pulmonary hypertension (PH), is…Abstract Number: 0775 • ACR Convergence 2025
Increased Circulating Microbial Small RNA tDR-1 is Associated with Decreased Progression to Future Clinical Rheumatoid Arthritis In High Risk Individuals and Reduces In Vitro Type 1 Interferon Response Gene Expression
Background/Purpose: Microbial small RNAs (sRNAs) can regulate human genes, often with anti-inflammatory effects. We previously showed that an increased plasma concentration of a tRNA-derived RNA…Abstract Number: 1777 • ACR Convergence 2025
Granzyme B as a regulator of interferon alpha production in cutaneous lupus erythematosus
Background/Purpose: Recent studies have shown that monocytes, macrophages, and conventional DCs, but not plasmacytoid dendritic cells (pDC), are major sources of IFNα in cutaneous lupus…Abstract Number: 0951 • ACR Convergence 2025
Epidermal IFNκ Increases Circulating and Cutaneous Monocytes in a C57/Bl6 Overexpression Mouse Model
Background/Purpose: Lupus is a heterogeneous inflammatory autoimmune disease affecting a variety of organs, including skin manifestations such as photosensitivity and cutaneous lesions. The skin of…Abstract Number: 0518 • ACR Convergence 2025
Deregulation of PSGL-1, HLA-DR and IFNα expression in peripheral innate immune cells of primary Sjögren Syndrome patients
Background/Purpose: Primary Sjögren's Syndrome (pSS) is an autoimmune disease characterized by lymphocytic infiltration of exocrine glands and systemic manifestations including cutaneous and renal involvement. Phagocytes--monocytes,…Abstract Number: L01 • ACR Convergence 2024
Targeted Exosite Inhibition of STING Activation of TBK1 Selectively Blocks Type I Interferon and NFκB Responses for Treatment of Autoimmune Diseases
Background/Purpose: The cGAS-TBK1-STING pathway senses nucleic acids for innate immunity. Aberrant activation of the pathway is linked to autoimmune diseases including Systemic and Cutaneous Lupus…Abstract Number: 0633 • ACR Convergence 2024
Novel LINE-1 Reverse Transcriptase Inhibitors Can Suppress Type I Interferon Responses and Are Promising Therapeutics for Lupus
Background/Purpose: Long Interspersed Element-1 (LINE-1) retrotransposon encodes for two proteins, ORF1p and ORF2p. ORF1p is a chaperone protein while ORF2p contains reverse transcriptase (RT) and…Abstract Number: 1537 • ACR Convergence 2024
Discovery of VENT-03: A Novel Clinical cGAS Inhibitor for the Treatment of SLE and Other Autoimmune Diseases
Background/Purpose: Cyclic GMP-AMP synthase (cGAS) is a nucleic acid sensor that plays a central role in anti-viral responses. Following the detection of intracellular double-stranded DNA,…Abstract Number: 1828 • ACR Convergence 2024
SSc Fibroblasts Trigger a Systemic Type I Interferon Response in SSc Patients Through Canonical TGF-β Receptor Signalling
Background/Purpose: Systemic Sclerosis is an autoimmune disease which is characterized by fibrosis of the skin and the internal organs with variable severity. Type I interferon…Abstract Number: 0666 • ACR Convergence 2024
Interferon-stimulated Genes on Peripheral CD8+ T Cells of SLE Patients Were the Keys for Early Response to BAFF/APRIL-targeted Therapy
Background/Purpose: Systemic lupus erythematosus (SLE) is a heterogeneous autoimmune disease. BAFF/APRIL-targeted therapy exert therapeutic effects through the inhibition of B-cell activating factor (BAFF) and a…Abstract Number: 1553 • ACR Convergence 2024
Safety, Pharmacokinetics, Clinical Efficacy and Exploratory Biomarker Results from a Randomized, Double-Blind, Placebo-Controlled Phase 1b Study of Enpatoran in Active Systemic and Cutaneous Lupus Erythematosus (SLE/CLE)
Background/Purpose: Enpatoran, a highly selective, potent and reversible dual toll-like receptor 7/8 (TLR7/8) inhibitor, targets key innate and adaptive immune processes involved in the pathogenesis…Abstract Number: 1831 • ACR Convergence 2024
Role of Long Non-Coding RNA H19 in Mediating Skin Fibrosis in Systemic Sclerosis
Background/Purpose: Systemic Sclerosis (SSc) is an immune driven condition leading to skin fibrosis, as well as internal organs. Dermal fibroblasts are the key cellular elements…
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