Abstracts tagged "interferon"

Abstract Number: 1170 • ACR Convergence 2022
Systemic Sclerosis Dermal Fibroblast-derived Exosomes Trigger a Type 1 Interferon Rresponse in Keratinocytes Through TBK1
Jessica Bryon¹, Christopher Wasson¹, Rebecca Ross¹, Elton Zeqiraj¹ and Francesco Del Galdo², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Elevated Type I IFN response is present in blood and affected tissues in systemic sclerosis (SSc) and correlates with disease activity and response to…
Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: 0641 • ACR Convergence 2022
IL-4 Receptor Signaling and IKAROS Transcriptomic Program Maintains Naïve B-cell Quiescence and Antagonizes SLE
John Mountz, Min Gao, Shanrun Lui, Walter Chatham and Hui-Chen Hsu, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Factors that promote B-cell quiescence and homeostasis at the transitional (Tr) and naive stages of B cells to prevent excessive TLR7 and type I…
Abstract Number: 1629 • ACR Convergence 2022
AUR200: An Improved BAFF/APRIL Inhibitor with Increased Potency and Safety for the Treatment of B Cell-Mediated Diseases
Shawn Morales, Jennifer Cross and Robert Huizinga, Aurinia Pharmaceuticals, Inc., Victoria, BC, Canada
Background/Purpose: AUR200 targets both B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), which are key mediators in the pathogenesis of B cell-mediated autoimmune…
Abstract Number: 2264 • ACR Convergence 2022
Dysregulated Interferon Gamma Signals Promote Systemic Autoimmunity in STAT1 Gain of Function Syndrome
Andrea Largent¹, Jane Buckner², David Rawlings¹ and Shaun Jackson¹, ¹Seattle Children's Research Institute, Seattle, WA, ²Benaroya Research Institute at Virginia Mason, Seattle, WA
Background/Purpose: Heterozygous gain-of-function mutations in STAT1 promote a complex disorder of immune dysregulation including chronic mucocutaneous candidiasis (CMC) and systemic autoimmunity. However, given widespread expression…
Abstract Number: 0644 • ACR Convergence 2022
Long-lived Plasma Cells in the Lupus Bone Marrow Are Imprinted by Interferon
Jennifer Barnas¹, Diana Alzamareh², Neha Nandedkar-Kulkarni², Jennifer Albrecht², Nida Meednu², Cameron Baker², Andrew McDavid¹ and Jennifer Anolik², ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
Background/Purpose: Systemic lupus erythematous (SLE) is a complex autoimmune disorder with heterogeneous disease presentation and a multi-pronged pathogenesis. Although autoreactive plasma cells (PC) play a…
Abstract Number: 1664 • ACR Convergence 2022
TRIM21 as a Regulator of UVB-Driven IFN Responses in Lupus
Richard Moore, Gantsetseg Tumurkhuu, Daniel Wallace and Caroline Jefferies, Cedars-Sinai Medical Center, Los Angeles, CA
Background/Purpose: Patients with systemic lupus erythematosus (SLE) experience photosensitivity where exposure to ultraviolet light B (UVB) drives lupus flares and systemic symptoms. Although the mechanisms…
Abstract Number: 2267 • ACR Convergence 2022
Plasmacytoid Dendritic Cells Are Not Major Producers of Type 1 Interferons in Cutaneous Lupus
Thomas Vazquez¹, Nilesh Kodali¹, DeAnna Diaz², Jay Patel³, Emily Keyes², Grant Sprow², Meena Sharma¹, Mariko Ogawa-Momohara², Madison Grinnell², Josh Dan² and Victoria Werth², ¹Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, ²Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, ³Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PN
Background/Purpose: Type 1 interferons (IFN-1) are major drivers of disease activity in systemic (SLE) and cutaneous lupus erythematosus (CLE). Plasmacytoid dendritic cells (pDCs) are the…
Abstract Number: 0008 • ACR Convergence 2022
CD11c+ T-Bet+ B Cells Traffic to Human Bone Marrow
Jennifer Barnas¹, Diana Alzamareh², Nida Meednu² and Jennifer Anolik², ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
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
Ruth Fernandez Ruiz¹, Ilona Nln², Jacqueline Paredes² and Timothy Niewold², ¹Hospital for Special Surgery/Weill Cornell Medicine, New York, NY, ²Hospital for Special Surgery, New York, NY
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
Shannon Estadt, Mehrnaz Gharaee-Kermani, Bin Xu, Tyson Moore, Andrew Hannoudi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
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
Merri-Grace Allred and Scott Lieberman, University of Iowa, Iowa City, IA
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
Shohei Nakamura, Yuko okamoto, Yasuhiro Katsumata and Masayoshi Harigai, Division of Rheumatology, Department of Internal Medicine, Tokyo Women’s Medical University School of Medicine, Tokyo, Japan
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
Jie An¹, Sladjana Skopelja-Gardner², Xizhang Sun¹, Lena Tanaka¹ and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Dartmouth Hitchcock Medical Center, Lebanon, NH
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
Lauren Covert¹, George Truskey² and Jeffrey Dvergsten¹, ¹Duke University Hospital, Durham, NC, ²Duke University, Durham, NC
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…

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