Abstract Number: 0068 • ACR Convergence 2020
Single Cell RNA-seq to Characterize Monocyte Subtypes in the Autoinflammatory Interferonopathy, SAVI and the Inflammasomopathy, NOMID
Background/Purpose: Monocytes are pivotal producers of key inflammatory cytokines that drive autoinflammatory diseases. In SAVI, constitutive STING activation causes chronic activation with increased type-I IFN…Abstract Number: 0946 • ACR Convergence 2020
ENPP1 Regulates UV Light Triggered Type I Interferon Response in the Skin
Background/Purpose: Systemic lupus erythematosus (SLE) patients have a prominent type I interferon (IFN-I) signature in both the lesional and non-lesional skin. We recently showed that…Abstract Number: 1818 • ACR Convergence 2020
IgG and IgA Autoantibodies Against L1 ORF1p Expressed in Granulocytes Correlate with Granulocyte Consumption and Disease Activity in Pediatric Systemic Lupus Erythematosus
Background/Purpose: To quantitate autoantibodies against the RNA-binding p40 (ORF1p) protein encoded by the L1 retroelement, expression of p40 itself, and markers of neutrophil death in…Abstract Number: 0074 • ACR Convergence 2020
The Intracellular DNA Sensor STING Protects Against Bone Loss Through Regulation of Type I Interferons
Background/Purpose: The intracellular DNA sensor Stimulator of Interferon Genes (STING) is essential for detection of viral and bacterial pathogen DNA. As with other pathways in…Abstract Number: 0957 • ACR Convergence 2020
Mitochondrial ROS as a Regulator of Calcinosis in Juvenile Dermatomyositis
Background/Purpose: Calcinosis, the accumulation of calcium crystals in soft tissues, is often a locus of infection and a debilitating manifestation of chronic juvenile dermatomyositis (JDM),…Abstract Number: 1827 • ACR Convergence 2020
Flare Reduction and Oral Corticosteroid Taper in Patients with Active SLE Treated with Anifrolumab in 2 Phase 3 Trials
Background/Purpose: Objectives of long-term SLE management are not only to reduce disease activity, but also to prevent flares and minimize exposure to oral corticosteroids (OCS),…Abstract Number: 0076 • ACR Convergence 2020
The Role of Interferon Kappa in Psoriasis
Background/Purpose: Psoriasis is a common, chronic inflammatory autoimmune skin diseases characterized by hyperproliferation and abnormal differentiation of keratinocytes and infiltration of inflammatory cells. Early infiltration…Abstract Number: 0986 • ACR Convergence 2020
BIIB059, a Humanized Monoclonal Antibody Targeting Blood Dendritic Cell Antigen 2 on Plasmacytoid Dendritic Cells, Shows Dose-Related Efficacy in a Phase 2 Study in Participants with Active Cutaneous Lupus Erythematosus
Background/Purpose: No approved targeted therapies have been developed for cutaneous lupus erythematosus (CLE), a disfiguring autoimmune disease that severely impairs quality of life.1 BIIB059 is…Abstract Number: 1828 • ACR Convergence 2020
Comprehensive Efficacy of Anifrolumab Across Organ Domains in Patients with Active SLE: Pooled Data from 2 Phase 3 Trials
Background/Purpose: SLE is a heterogeneous autoimmune disease with clinical manifestations across multiple organ systems. In the phase 3 TULIP-1 and TULIP-2 trials, anifrolumab treatment resulted…Abstract Number: 0171 • ACR Convergence 2020
Interferon Response Gene Expression Differs in Whole Blood, Peripheral Blood Mononuclear Cells, Monocytes, T Cells, B Cells, and NK Cells in Patients with the Autoinflammatory Interferonopathies, CANDLE and SAVI
Background/Purpose: The disease progression of patients (pts.) with type-I interferon (IFN)-mediated diseases undergoing treatment with JAK1 and JAK2 inhibitors is monitored in part by measuring…Abstract Number: 0990 • ACR Convergence 2020
Interferon Lambda Promotes Human Plasma Cell Differentiation in Lupus and Healthy Donors
Background/Purpose: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease characterized by antinuclear autoantibodies produced by plasma cells. Type I interferon (IFN) are cytokines which…Abstract Number: 1834 • ACR Convergence 2020
Biomarker Analysis of IFN-I Modulation in JNJ-839: First-in-Human Study for Systemic Lupus Erythematosus
Background/Purpose: JNJ-839 is a fully human neutralizing antibody selective to human IFN-ω and IFN-α subtypes. Here we report the exploratory biomarker analyses of a Phase…Abstract Number: 0282 • ACR Convergence 2020
Expression of the cGAMP Transporter SLC19A1 Is Altered in Systemic Lupus Erythematosus
Background/Purpose: Inappropriate sensing of nucleic acids leading to enhanced type I interferon (IFN) induction is a hallmark of SLE, contributing to breakdown of immune tolerance…Abstract Number: 0992 • ACR Convergence 2020
Impact of Selective Inhibitors of Nuclear Export on SLE Plasma Cells Is Modulated by the BM Microenvironment
Background/Purpose: Systemic lupus erythematous (SLE) is a complex autoimmune disorder with heterogeneous disease presentation and a multi-pronged pathogenesis. Although autoreactive plasma cells play a key…Abstract Number: 1949 • ACR Convergence 2020
CB2 Receptor Distribution and Effects of LenabasumTM in Dermatomyositis In Vitro
Background/Purpose: Dermatomyositis (DM) patients report poor quality of life due to disease activity and persistent itch. Lenabasum is an oral non-immunosuppressive, non-psychoactive cannabinoid type 2…