Abstract Number: 2448 • ACR Convergence 2023
UV Light Exposure Induces a Type I Interferon Dependent Activation and Migration of Inflammatory Dendritic Cells to Local Lymph Nodes
Background/Purpose: Photosensitivity occurs in ~ 75% of lupus patients. Although ultraviolet (UV) light stimulates Type I interferon (IFN-I) in the skin, why lupus patients are…Abstract Number: 0724 • ACR Convergence 2023
Synovial Macrophage Subsets Defined by ScRNAseq Demonstrate Sexually Dimorphic Gene Expression in RA and a Mouse Inflammatory Arthritis Model
Background/Purpose: Macrophages (MФ) play a key pathogenic role in Rheumatoid Arthritis (RA), a disease that exhibits female sex bias. Recent work using scRNAseq has defined…Abstract Number: 1508 • ACR Convergence 2023
Predicting in Virtual Patients the Efficacy of an Anti IFNa Mab in Cutaneous Lupus Erythematosus
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a heterogeneous autoimmune disease that requires more specific treatments to target patient subsets. To support precision medicine approaches, we…Abstract Number: 2576 • ACR Convergence 2023
Rapid Onset of Response in Adult Dermatomyositis Patients Receiving Anti-interferon β (PF-06823859): Results of a Phase 2, Double-blind, Randomized, Placebo-Controlled Study
Background/Purpose: Interferon (IFN) dysregulation is a key feature in the pathogenesis of Dermatomyositis (DM). PF-06823859 is a potent, selective, humanized IgG1 neutralizing antibody directed against…Abstract Number: 0728 • ACR Convergence 2023
Systemic Lupus Erythematosus B Lymphocyte Responsiveness to Type I and Type III Interferon Is Determined by Donor IFN Status and B Cell Phenotype
Background/Purpose: Systemic lupus erythematous (SLE) is a heterogenous autoimmune disorder characterized by pathogenic antinuclear antibodies. An interferon (IFN) gene signature and B cell aberrations are…Abstract Number: 1580 • ACR Convergence 2023
CXCL13+ T Cell Differentiation in Systemic Lupus Erythematosus Is Controlled by Opposing Effects of Aryl Hydrocarbon Receptor and Type I Interferon
Background/Purpose: Expansion of B cell-helper T cells including T follicular helper (Tfh) and T peripheral helper (Tph) cells is a prominent feature of systemic lupus…Abstract Number: 0733 • ACR Convergence 2023
Global Identification of Lupus Genetic Risk Variants Facilitating the Type I Interferon Pathway Through CRISPR-based Genomic Screening
Background/Purpose: Genome-Wide Association Studies (GWAS) have unveiled over 1000 risk variants for lupus, predominantly situated in non-coding genomic regions. Their functional roles, especially their potential…Abstract Number: 1586 • ACR Convergence 2023
Keratinocyte VISTA Suppresses Skin IFN-I Production by Regulating DNA Damage Repair and Cytosolic DNA Sensing
Background/Purpose: Persistent production of type I interferons (IFN-Is) is one of the hallmarks of lupus skin disease that is exacerbated by ultraviolet (UV) light. We…Abstract Number: 023 • 2023 Pediatric Rheumatology Symposium
Effect of Type 1 Interferons and JAK Inhibitors on Gene Expression in Bioengineered Pediatric Skeletal Muscle
Background/Purpose: Genetic studies of new-onset juvenile dermatomyositis (JDM) exhibit elevation of Type 1 interferons (IFN 1) IFNα and IFNβ in blood, skin, and muscle. To…Abstract Number: 075 • 2023 Pediatric Rheumatology Symposium
Emapalumab Treatment Followed by Hematopoietic Stem Cell Transplantation in Systemic Juvenile Idiopathic Arthritis Complicated by Recurrent Macrophage Activation Syndrome
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of different rheumatic diseases, particularly of systemic juvenile idiopathic arthritis (sJIA).Methods: We report the case of…Abstract Number: 080 • 2023 Pediatric Rheumatology Symposium
Analysis of Proteasomal Activity – a Potential Diagnostic Tool for Proteasome-associated Autoinflammatory Syndromes (PRAAS)
Background/Purpose: Interferonopathies are a recently recognized group of genetic syndromes associated with uncontrolled activation of interferon. PRAAS (proteasome-associated autoinflammatory syndromes) is an interferonopathy caused by…Abstract Number: 0008 • ACR Convergence 2022
CD11c+ T-Bet+ B Cells Traffic to Human Bone Marrow
Background/Purpose: CD11c+ age/autoimmunity-associated B cells (ABC) are associated with autoantibody producing cells and disease flare in systemic lupus erythematosus. ABC were initially identified in aged…Abstract Number: 0646 • ACR Convergence 2022
Modulation of Type I IFN Production and Regulatory T Cells by the PKGI/ROCK Signaling Pathway
Background/Purpose: IFNα is a pathogenic factor in SLE. The PRKG1 rs7897633 variant has been previously identified as the top hit in European ancestry patients with…Abstract Number: 1692 • ACR Convergence 2022
Interferon Alpha Promotes Ultraviolet Light-Mediated Keratinocyte Apoptosis in a Caspase-8 Dependent Manner via Upregulation of Interferon Regulatory Factor 1
Background/Purpose: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by enhanced sensitivity to ultraviolet radiation (UVR). UVR can trigger cutaneous and systemic disease…Abstract Number: 0012 • ACR Convergence 2022
Role of Type L IFN Signaling on B Cell Pathogenicity in Early Sjögren’s Disease in Mouse Model
Background/Purpose: Sjogren's disease is a chronic autoimmune disease autoimmune disease characterized by foci of inflammation of the lacrimal and salivary glands. As disease progresses, B…
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