Abstracts tagged "interferon"

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…
Abstract Number: 0657 • ACR Convergence 2022
DB-2304, a Novel anti-BDCA2 Monoclonal Antibody Drug Conjugate, Displayed Great Potency in Suppression of pDC Functions
Xi Li, Yu Zhang, Bing Li and Haiqing Hua, Duality Biologics, Shanghai, China
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
Ildefonso Sánchez Cerrillo¹, Marta Calvet Mirabent¹, Ana Triguero², Diego Calzada Fraile³, Mariel Valdivia¹, Marta Ramírez³, Enrique Vázquez de Luis³, Alberto Benguría Filippini³, Roberto Moreno³, María Magdalena Adrados de llano¹, Hortensia de la Fuente¹, Ilya Tsukalov⁴, Emilia Roy Vallejo⁵, Almudena Ramiro³, Salvador Iborra⁶, Francisco Sánchez Madrid¹, Ana Dopazo³, Isidoro Gonzalez⁷, Santos Castañeda⁸ and Enrique Martín Gayo⁴, ¹Hospital La Princesa, Madrid, Spain, ²Hospital Universitario La Princesa, Madrid, Spain, ³Centro de Investigaciones Cardiovasculares, Madrid, Spain, ⁴Universidad Autónoma de Madrid, Madrid, Spain, ⁵Internal Medicine Department, Hospital Universitario La Princesa, Instituto de Investigación Sanitaria La Princesa (IIS-IP), Madrid, Spain, ⁶Universidad Complutense de Madrid, Madrid, Spain, ⁷Hospital Universitario de La Princesa, Madrid, Spain, ⁸Division of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain
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
Rohit Aggarwal¹, Brendan Johnson², Jolie Feldman³, Austin Gromatzky⁴ and Paul N Mudd Jr.², ¹Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ²Priovant Therapeutics, Durham, NC, ³Priovant Therapeutics, New York, NY, ⁴Priovant Therapeutics, Washington, DC
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
Lisa Abernathy-Close, Suzanne Shoffner-Beck, Annie Lu, Amanda Victory, Amy Hurst, Craig Dobry, Rachael Wasikowski, Johann Gudjonsson, Alex Tsoi and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
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…
Abstract Number: 1711 • ACR Convergence 2022
The Cellular Basis for Type I Interferon Production Following Ultraviolet Light Stimulated Cyclic-GMP-AMP Synthase Activation in the Skin
Jie An, Xizhang Sun, Lena Tanaka and Keith Elkon, University of Washington, Seattle, WA
Background/Purpose: SLE patients characteristically have a type I interferon (IFN-I) signature in peripheral blood cells and this same signature is prominent in lesional and non-lesional…
Abstract Number: L20 • ACR Convergence 2021
Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)
Fabrizio De Benedetti¹, Alexei Grom², Paul Brogan³, Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci¹, Despina Eleftheriou³, Charalampia Papadopoulou³, Pierre Quartier⁴, Jordi Antón⁵, Rikke Frederiksen⁶, Veronica Asnaghi⁶ and Cristina De Min⁶, ¹Bambino Gesù Children's Hospital IRCCS, Rome, Italy, ²Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁵Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, ⁶Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…

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