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…Abstract Number: 0652 • ACR Convergence 2022
Serum from Patients with SLE and Anti-MDA5 Antibody-Positive Dermatomyositis Induce Shared Monocyte Cytokine Signature Through Type 1 Interferon Pathway
Background/Purpose: Although type 1 interferon (IFN) plays a pivotal role in the pathogenesis of systemic lupus erythematosus (SLE) and polymyositis/dermatomyositis (PM/DM) [1] as typically evidenced…Abstract Number: 1693 • ACR Convergence 2022
Skin Pigmentation Association with Regulation of Cyclic-GMP-AMP Following Skin Exposure to UV Light
Background/Purpose: Amongst the most important cytosolic DNA sensors is Cyclic GMP-AMP synthase (cGAS). Binding of DNA to cGAS results in the synthesis of the cyclic…Abstract Number: 0041 • ACR Convergence 2022
Modeling Juvenile Dermatomyositis with Engineered Human Skeletal Muscle: Effects of Type I Interferonβ and Janus Kinase Inhibitors
Background/Purpose: Upregulation of Type I interferons (IFN I), including IFNβ, is a hallmark of adult and juvenile dermatomyositis (JDM), but its role in pathogenesis is…Abstract Number: 0657 • ACR Convergence 2022
DB-2304, a Novel anti-BDCA2 Monoclonal Antibody Drug Conjugate, Displayed Great Potency in Suppression of pDC Functions
Background/Purpose: BDCA2 (Blood Dendritic Cell Antigen 2) is specifically expressed on plasmacytoid dendritic cells (pDCs), a type I interferon-producing cells, which have been implicated in…Abstract Number: 1703 • ACR Convergence 2022
Potential Regulation of NK Cells by CD1c+ Dendritic Cells Through RIG-I/DDX60 Pathway Involved in Th17 Responses in Primary Sjögren Syndrome
Background/Purpose: Altered Th17, CD8+ T cell and B cell adaptative immune responses in patients with primary Sjögren syndrome (pSS) have been characterized in salivary glands…Abstract Number: 0157 • ACR Convergence 2022
Brepocitinib for the Treatment of Dermatomyositis: Pharmacologic and Clinical Rationale
Background/Purpose: Brepocitinib is a novel, orally available, TYK2/JAK1 inhibitor in Phase 3 development for the treatment of dermatomyositis (DM), a chronic immune-mediated disease of the…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…
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