Abstract Number: 168 • 2020 Pediatric Rheumatology Symposium
Examining the Role of IFN-I and Langerhans Cell ADAM17 in Lupus Photosensitivity
Background/Purpose: Photosensitivity resulting in inflammatory skin lesions is a hallmark of cutaneous lupus. Lesions can be disfiguring and have a negative impact on quality of…Abstract Number: 005 • 2020 Pediatric Rheumatology Symposium
Single Cell Sequencing of the Skin to Define Cell Populations of Interest in Localized Scleroderma (LS)
Background/Purpose: Scleroderma is an autoimmune disorder involving inflammatory driven fibrosis, which encompasses systemic sclerosis (SSc) and localized scleroderma (LS). LS and SSc share histological characteristics,…Abstract Number: 027 • 2020 Pediatric Rheumatology Symposium
Clinical Features and Outcomes in Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE) Syndrome: Before and After JAK-inhibition
Background/Purpose: Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy And Elevated Temperature (CANDLE) Syndrome is an autoinflammatory interferonopathy caused by mutations in the genes encoding for components…Abstract Number: 079 • 2020 Pediatric Rheumatology Symposium
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Background/Purpose: Macrophage Activation Syndrome (MAS) and Secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm. Prompt recognition and early treatment are essential…Abstract Number: 080 • 2020 Pediatric Rheumatology Symposium
Therapeutic Interferon Gamma Neutralization with Emapalumab in Patients with NRLC4- and CDC42-Associated Diseases Characterized by Recurrent and Severe Hemophagocytic Lymphohistiocytosis
Background/Purpose: Interferon gamma (IFNγ) is a pivotal mediator of HLH. Emapalumab (a fully human anti-IFNγ monoclonal antibody) is efficacious in patients with primary HLH. In…Abstract Number: 140 • 2020 Pediatric Rheumatology Symposium
Serum Biomarkers in a German Cohort of Patients with Systemic Juvenile Idiopathic Arthritis and Their Relationship to Response to Interleukin-1 Blockade
Background/Purpose: Most, but not all, patients with systemic juvenile idiopathic arthritis (systemic JIA) respond to therapy with interleukin (IL)-1 blocking agents but predictive factors have…Abstract Number: L06 • 2019 ACR/ARP Annual Meeting
Interferon-gamma (IFN-γ) Neutralization with Emapalumab and Time to Response in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (s-JIA) who failed High-Dose Glucocorticoids
Background/Purpose: MAS is a severe complication of rheumatic diseases, most frequently sJIA and adult-onset Still’s disease. It is characterized by fever, hepatosplenomegaly, liver dysfunction, cytopenias,…Abstract Number: 2026 • 2019 ACR/ARP Annual Meeting
MicroRNA-27a Can Contribute to Interferon Signatures in Systemic Lupus Erythematosus via the Suppression of Tripartite Motif-containing Protein 27
Background/Purpose: Type Ⅰ interferons (IFN) contribute to antiviral innate immune responses. Upon viral infection, pattern recognition receptors trigger TANK-binding kinase 1 (TBK1) activation and lead…Abstract Number: 2032 • 2019 ACR/ARP Annual Meeting
Enhanced IFN a Production and STING Pathway in Monocytes in Systemic Lupus Erythematosus Is Suppressed by the Inhibition of mTOR Activation
Background/Purpose: Interferona (IFNa) is increased and plays an important role in the pathogenesis of systemic lupus erythematosus (SLE). Overexpression of type I IFN regulated genes…Abstract Number: 2033 • 2019 ACR/ARP Annual Meeting
IRAK4 Inhibition Suppresses TLR7, TLR9, and SLE Serum-Induced IFNA Production in Primary Human Plasmacytoid Dendritic Cells
Background/Purpose: A hallmark of lupus is the presence of antinuclear autoantibodies, including those against RNA-protein complexes and double-stranded DNA (dsDNA).1,2 FcγR-mediated internalization of these nucleic…Abstract Number: 2563 • 2019 ACR/ARP Annual Meeting
PK/PD, Safety and Exploratory Efficacy of Subcutaneous Anifrolumab in SLE: A Phase-II Study in Interferon Type I High Patients with Active Skin Disease
Background/Purpose: Anifrolumab, a fully human anti–IFN Type I receptor mAb, is under investigation for the treatment of SLE at a dose of 300 mg intravenously…Abstract Number: 2739 • 2019 ACR/ARP Annual Meeting
Skin Disease Activity and Autoantibody Phenotype Are Major Determinants of Blood Interferon Signatures in Dermatomyositis
Background/Purpose: Interferon (IFN) signaling is upregulated in dermatomyositis (DM) and thought to play a role in pathogenesis. An IFN gene signature in peripheral blood of…Abstract Number: 78 • 2019 ACR/ARP Annual Meeting
Ultraviolet Light Induces Increased T Cell Activation in Lupus-Prone Mice via Type I Interferon-Dependent Inhibition of T Regulatory Cells
Background/Purpose: Ultraviolet (UV) light is a known trigger of skin and possibly systemic inflammation in systemic lupus erythematosus (SLE) patients. Although type I interferons (IFN)…Abstract Number: 2808 • 2019 ACR/ARP Annual Meeting
Linking Toll-Like Receptor Signaling and Type I Interferons to Inflammation and Fibrosis in a Macrophage/Fibroblast Model of Congenital Heart Block
Background/Purpose: Since one of the strongest associations with antibodies (abs) to SSA/Ro (Ro60) is the development of congenital heart block (CHB), this model provides an…Abstract Number: 88 • 2019 ACR/ARP Annual Meeting
Type I Interferon Signature Activation in Antiphospholipid Syndrome: Gene Expression Heterogeneity Among Disease Subsets
Background/Purpose: Type I Interferons (IFN) play a key role in the pathogenesis and evolution of various autoimmune diseases. Previous studies have demonstrated that the expression…
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