Abstracts tagged "interferon"

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
Eric Morand¹, Richard Furie², Ian Bruce³, Ed Vital⁴, Maria Dall'Era⁵, Emmanuelle Maho⁶, Lilia Pineda⁷ and Raj Tummala⁷, ¹Monash University, Melbourne, Australia, ²Zucker School of Medicine at Hofstra/Northwell, Great Neck, ³Centre for Epidemiology Versus Arthritis, The University of Manchester and NIHR Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom, ⁴University of Leeds; NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom, ⁵Division of Rheumatology, University of California, San Francisco, CA, ⁶BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁷BioPharmaceuticals R&D, AstraZeneca, Gaithersburg
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
Jacob Mitchell¹, Sara Alehashemi², Bernadette Marrero³, Yan Huang⁴, Sofia Torreggiani¹, Lena Bichell¹, Gina Montealegre Sanchez⁵, Raphaela Goldbach-Mansky⁶ and Adriana de Jesus⁷, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³Computational Systems Biology Section/NIAID/NIH, Bethesda, MD, ⁴NIH, Bethesda, ⁵NIAID/NIH, Rockville, MD, ⁶Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD, ⁷Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD
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
Jennifer Barnas¹, Jennifer Albrecht¹ and Jennifer Anolik¹, ¹University of Rochester Medical Center, Rochester, NY
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
Ashley Orillion¹, Loqmane Seridi¹, Matteo Cesaroni², Jessica Schreiter¹, Jacqueline Benson³, William Stohl⁴, Walter Winn Chatham⁵, Richard Alan Furie⁶, Thi-Sau Migone⁷, Stanley Marciniak¹, Zhenling Yao¹, Bhaskar Srivastava¹, Marc Chevrier² and Jarrat Jordan¹, ¹Janssen Research & Development, LLC, Spring House, PA, ²Janssen Research & Development, LLC, Spring House, ³Janssen Research & Development, LLC, South San Francisco, CA, ⁴University of Southern California Keck School of Medicine, Los Angeles, CA, ⁵University of Alabama at Birmingham, Birmingham, AL, ⁶Feinstein Institutes for Medical Research, Northwell Health, Manhasset, NY, ⁷Independent Consultant, Spring House
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
Jeong Min Yu¹, Gantsetseg Tumurkhuu², Erica Montano², Gabriela de los Santos², Daniel J Wallace², Mariko Ishimori³ and Caroline Jefferies¹, ¹Cedars-Sinai Medical Center, West Hollywood, CA, ²Cedars-Sinai Medical Center, Los Angeles, ³Cedars-Sinai Medical Center, Los Angeles, CA
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
Neha Nandedkar-Kulkarni¹, Nida Meednu², Jennifer Albrecht², Jennifer Barnas², Douglas Widman³ and Jennifer Anolik², ¹University of Rochester, Rochester, NY, ²University of Rochester Medical center, Rochester, NY, ³Karyopharm Therapeutics, Newton, MA
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
Spandana Maddukuri¹, Jay Patel², Christina Bax³, Maria Wysocka³ and Victoria Werth⁴, ¹University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, ²University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, ³University of Pennsylvania, Department of Dermatology, Philadelphia, ⁴University of Pennsylvania and Corporal Michael J. Crescenz Veterans Administration Hospital, Philadelphia
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…
Abstract Number: 0284 • ACR Convergence 2020
Oxidative DNA Damage Accelerates Skin Inflammation in Pristane-induced Lupus Model
Gantsetseg Tumurkhuu¹, Shuang Chen², Erica Montano¹, Malcolm Lane², Michifumi Yamashita², Janet Markman², Luz Blanco³, Mariana Kaplan⁴, Kenichi Shimada², Timothy Crother², Mariko Ishimori², Daniel J Wallace¹, Caroline Jefferies⁵ and Moshe Arditi², ¹Cedars-Sinai Medical Center, Los Angeles, ²Cedars-Sinai Medical Center, Los Angeles, CA, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases, Centreville, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁵Cedars-Sinai Medical Center, West Hollywood, CA
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a chronic inflammatory autoimmune disease in which type I interferons (IFN) play a key role. The IFN response can…
Abstract Number: 1067 • ACR Convergence 2020
Anti-Viral Proinflammatory Phenotype in Circulating Monocytes from Patients with Anti-Melanoma Differentiation-Associated Gene 5 Antibody-Associated Interstitial Lung Disease
Takahisa Gono¹, Yuka Okazaki¹ and Masataka Kuwana², ¹Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Japan, ²Department of Allergy and Rheumatology, Nippon Medical School Graduate School of Medicine, Tokyo, Tokyo, Japan
Background/Purpose: Anti-melanoma differentiation-associated gene 5 (MDA5) antibody is associated with interstitial lung disease (ILD), which often represents rapidly progressive course and fatal outcomes. Circulating levels…
Abstract Number: 0286 • ACR Convergence 2020
An SLE-linked ITGAM Gene Variant Changes Mac-1 Structure, Signaling, and Surface Expression and Enhances IFNg Production and Antigen Presentation by B Cells
Joseph Blake¹, Alexander Szalai², Jeffrey Edberg³ and James Mobley³, ¹UAB, Birmingham, ²University of Alabama at Birmingham, birmingham, AL, ³UAB, Birmingham, AL
Background/Purpose: SLE is a chronic and debilitating disease; in the USA with an estimated incidence of 3-10 per 100,000 people and currently affecting an estimated…
Abstract Number: 1150 • ACR Convergence 2020
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Arianna De Matteis¹, Denise Pires Marafon¹, Ivan Caiello¹, Manuela Pardeo¹, Giulia Marucci¹, Emanuela Sacco¹, Giusi Prencipe¹, Fabrizio De Benedetti² and Claudia Bracaglia¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ²Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) and secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm, in which IFNγ plays a pivotal role.…
Abstract Number: 0287 • ACR Convergence 2020
RNA Externalized by Neutrophil Extracellular Traps Promotes Inflammatory Pathways in Endothelial Cells
Xinghao Wang¹, Philip Carlucci², Jorge Romo-Tena¹, Jose Torres-Ruiz¹, Hong-Wei Sun¹, Markus Hafner¹, Mariana Kaplan³ and Luz Blanco⁴, ¹NIAMS, National Institute of Health, Bethesda, ²New York University School of Medicine, New York, NY, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Centreville
Background/Purpose: Neutrophil extracellular traps (NETs) are extracellular lattices composed of nucleic material bound to neutrophil granule proteins. NETs may play pathogenic roles in development and…
Abstract Number: 1158 • ACR Convergence 2020
Clinical Features and Outcomes in STING-Associated Vasculopathy with Onset in Infancy (SAVI)
Sofia Torreggiani¹, Sara Alehashemi², Jacob Mitchell¹, Gema Souto Adeva¹, Bin Lin¹, Jenna Wade¹, Gina Montealegre Sanchez³, Abdulrahman Alrasheed⁴, Sibel Balci⁵, Roberta Berard⁶, Borzutzky Arturo⁷, Jürgen Brunner⁸, Bjoern Buehring⁹, Al Adba Buthaina¹⁰, Caterina Cancrini¹¹, John Carter¹², Mireia Corbeto Lopez¹³, Fabrizio De Benedetti¹⁴, Huy Do¹⁵, Gregor Dueckers¹⁶, Les Folio¹⁵, Antonella Insalaco¹⁷, Rabia Miray Kisla Ekinci⁵, Michael Miller¹⁸, Marco Montes Cano¹⁹, Marie-Paule Morin²⁰, Seza Ozen²¹, Lucia Pacillo¹¹, Suzanne Ramsey²², Adam Reinhardt²³, Dax Rumsey²⁴, Laisa Santiago²⁵, Grant Schulert²⁶, Benjamin Wright²⁷, Adriana de Jesus²⁸ and Raphaela Goldbach-Mansky²⁹, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Clarksville, MD, ³NIAID/NIH, Rockville, MD, ⁴King Abdullah Specialized Children Hospital, Riyadh, Riyadh, Saudi Arabia, ⁵Department of Pediatric Rheumatology, Cukurova University Faculty of Medicine, Adana, Turkey, ⁶London Health Sciences Centre, London, ON, Canada, ⁷Pontificia Universidad Católica de Chile, Santiago, Chile, ⁸Tirol Kliniken, Innsbruck, Innsbruck, Austria, ⁹Rheumazentrum Ruhrgebiet, Ruhr-University-Bochum, Herne, Germany, ¹⁰Sidra Medicine, Doha, Doha, Qatar, ¹¹Unit of Immune and Infectious Diseases, Scientific Institute for Research and Healthcare (IRCCS) Childrens’ Hospital Bambino Gesù, University Department of Pediatrics (DPUO); Department of Systems Medicine, University of Rome Tor Vergata, Roma, Italy, ¹²University of South Florida, Tampa, FL, ¹³Vall d’Hebron Hospital Universitari, Vall d’Hebron Barcelona Hospital Campus, Barcelona, Spain, ¹⁴Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁵Radiology and Imaging Sciences, Clinical Center, NIH, Bethesda, ¹⁶Helios Kliniken - Kinderklinik, HELIOS Klinikum Krefeld, Germany, Krefeld, Germany, ¹⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ¹⁸Feinberg School of Medicine, Northwestern University Ann & Robert H. Lurie Children’s Hospital of Chicago, Chicago, IL, ¹⁹Hospital Universitario Virgen del Rocío, Sevilla, Sevilla, Spain, ²⁰Université de Montréal, CHU Sainte-Justine, Montréal, Canada, ²¹Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, Ankara, Turkey, ²²IWK Health Centre, Dalhousie University, Halifax, NS, Canada, ²³Boys Town National Research Hospital, Omaha, Omaha, NE, ²⁴Alberta Health Services – Edmonton Zone (Stollery Children’s Hospital), University of Alberta, Edmonton, AB, Canada, ²⁵Johns Hopkins All Children's Hospital, St. Petersburg, FL, ²⁶PRCSG, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²⁷Mayo Clinic, Phoenix, AZ, ²⁸Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Silver Spring, MD, ²⁹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is an autoinflammatory interferonopathy caused by gain-of-function mutations in STING1, characterized by peripheral vasculopathy and interstitial lung…
Abstract Number: 0289 • ACR Convergence 2020
Endogenous Interferon-β and Low IL-4R on Transitional B Cells Promotes Lupus Nephritis
Fatima Alduraibi¹, Huma Fatima¹, W. Winn Chatham¹, Hui-Chen Hsu¹ and John Mountz², ¹University of Alabama at Birmingham, Birmingham, AL, ²University Alabama at Birmingham and Birmingham VA Medical Center, Birmingham, AL
Background/Purpose: We previously showed that B-cell endogenous interferon-beta (IFNβ) at the transitional (Tr) stage correlates with development of anti-Smith (anti-Sm) and renal disease as well…
Abstract Number: 1159 • ACR Convergence 2020
Novel STING1 Mutations Including in the Transmembrane Linker Region Cause STING-associated Vasculopathy with Onset in Infancy (SAVI)
Bin Lin¹, Dana Kahle¹, Adriana Almeida de Jesus¹, Sofia Torreggiani², Jacob Mitchell², Alexander Aue¹, Zheng Ji³, Tengchuan Jin³ and Raphaela Goldbach-Mansky⁴, ¹Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ³University of Science and Technology of China, Hefei, China (People's Republic), ⁴Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Potomac, MD
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by gain-of-function (GOF) mutations in STING1/TMEM173 that encodes stimulator of interferon genes,…

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