Abstracts tagged "interferon"

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,…
Abstract Number: 0292 • ACR Convergence 2020
Exhausted pSTAT5-IFNα Signaling Pathways in SLE Patients Are Correlated with Age-associated B Cells and Disease Activity
Samantha Slight-Webb¹, Miles Smith², Kevin Thomas¹, Susan Macwana¹, Holden Maecker³, Paul Utz⁴, Judith James⁵ and Joel Guthridge¹, ¹Oklahoma Medical Research Foundation, Oklahoma City, OK, ²Oklahoma Medical Research Foundation, Oklahoma City, ³Institute for Immunity, Transplantation and Infection, Stanford University School of Medicine, Stanford, CA, ⁴Stanford University School of Medicine, Stanford, CA, ⁵Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation;Department of Pathology, University of Oklahoma Health Sciences Center;Department of Medicine, University of Oklahoma Health Sciences Center, Edmond, OK
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by periods of elevated and suppressed clinical symptoms. Specific cell subsets, such as CD11c+ age-associated…
Abstract Number: 1399 • ACR Convergence 2020
Differential Impacts of TNFa Inhibitors on the Expression of Th Cytokines
Ching-Huang Ho¹, Andrea Silva¹ and I-Cheng Ho², ¹Brigham and Women's Hospital, Boston, MA, ²Birgham and Women's Hospital, Boston, MA
Background/Purpose: Inhibition of TNFα has emerged as an effective therapeutic approach for many autoimmune/inflammatory diseases.While the efficacy and safety profile of the five FDA-approved TNFis…
Abstract Number: 0297 • ACR Convergence 2020
Towards a Glucocorticoid Exposure Signature in SLE: Effects of Type I Interferon
Melissa Northcott¹, Linden Gearing², Hieu Nim³, Champa Nataraja³, Sarah Jones¹ and Eric Morand⁴, ¹Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Victoria, Australia, ²Hudson Institute of Medical Research, Clayton, Victoria, Australia, ³Monash University, Clayton, Victoria, Australia, ⁴Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Australia
Background/Purpose: Glucocorticoids (GC), utilised in SLE for their broad immunosuppressive actions, predominantly mediate these effects by interaction with the cytoplasmic GC receptor (GR) to modulate…
Abstract Number: 1405 • ACR Convergence 2020
Evaluating the Cellular Composition of Anti-synthetase Syndrome and Dermatomyositis Skin Lesions Using Image Mass Cytometry
Jay Patel¹, Adarsh Ravishankar¹, Spandana Maddukuri², Christina Bax³ and Victoria Werth⁴, ¹University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, ²University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Montville, NJ, ³University of Pennsylvania, Department of Dermatology, Philadelphia, ⁴University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, PA
Background/Purpose: Antisynthetase syndrome (AS) is a systemic autoimmune disorder characterized by the presence of anti-aminoacyl-tRNA synthetase antibodies, myositis, interstitial lung disease (ILD), mechanics hands, and…
Abstract Number: 0304 • ACR Convergence 2020
Type I Interferon Inhibits Glucocorticoid-Induced Leucine Zipper (GILZ) Expression and Upregulation by Glucocorticoids
Wendy Dankers¹, Melissa Northcott², Taylah Bennett³, Brendan Russ³, Jacqueline Flynn¹, Sarah Jones² and Eric Morand¹, ¹Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Australia, ²Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Victoria, Australia, ³Department of Microbiology, Monash University, Melbourne, Australia
Background/Purpose: Glucocorticoids (GC) are broadly used in the treatment of inflammatory diseases, including systemic lupus erythematosus (SLE). Despite their widespread use, most SLE patients do…
Abstract Number: 1443 • ACR Convergence 2020
High-dimensional Analyses of Checkpoint-inhibitor Related Arthritis Synovial Fluid Cells Reveal a Unique, Proliferating CD38hi Cytotoxic CD8 T Cell Population Induced by Type I IFN
Runci Wang¹, Karmela Kim Chan², Amy Cunningham-Bussel¹, Gregory Vitone³, Aidan Tirpack², Caroline Benson², Gregory Keras⁴, Anna Helena Jonsson⁵, Michael Brenner⁵, Laura Donlin⁶, Anne Bass⁷ and Deepak Rao¹, ¹Brigham and Women's Hospital, Boston, MA, ²Hospital For Special Surgery, New York, NY, ³Hospital for Special Surgery, New York, ⁴Brigham and Women’s Hospital, Division of Rheumatology, Inflammation, and Immunity, Boston, MA, ⁵Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁶Hospital for Special Surgery, Weill Cornell Medicine, New York, ⁷Hospital for Special Surgery/Weill Cornell Medicine, New York, NY
Background/Purpose: Checkpoint inhibitors (CI) used to treat cancer frequently trigger immune-related adverse events, including inflammatory arthritis. CI-related arthritis (CIrA) occurs in ~5% of treated patients,…
Abstract Number: 0462 • ACR Convergence 2020
Lupus-like Autoimmunity and Increased Interferon Response in Patients with STAT3-deficient Hyper-IgE Syndrome
Brian Dizon¹, Rishi Goel², Shuichiro Nakabo², Amanda Urban², Meryl Waldman², Lilian Howard², Dirk Darnell², Munir Buhaya², Sarfaraz Hasni³, Mariana Kaplan⁴, Alexandra Freeman² and Sarthak Gupta¹, ¹National Institutes of Health, BETHESDA, MD, ²National Institutes of Health, Bethesda, ³Lupus Clinical Trials Unit, NIAMS/NIH, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Autosomal dominant hyper-IgE syndrome (AD-HIES), also known as Job’s syndrome, is a rare primary immunodeficiency caused by dominant-negative loss-of-function (LOF) mutations in signal transducer…
Abstract Number: 1788 • ACR Convergence 2020
Assessment of the Impact of Interferon Levels on Cognitive Dysfunction in Patients with SLE
Andrew Kwan¹, Joan Wither², Juan Pablo Diaz-Martinez², Robin Green³, Dorcas Beaton⁴, Mahta Kakvan², Lesley Ruttan³, Carmela Tartaglia⁵, Marvin Fritzler⁶, May Choi⁷, Jiandong Su², Dennisse Bonilla², Nicole Anderson², Patricia Katz⁸ and Zahi Touma⁹, ¹University of Toronto, Department of Medicine, Toronto, ON, Canada, ²University of Toronto Lupus Clinic, Centre for Prognosis Studies in Rheumatic Diseases, Toronto Western Hospital, University Health Network, Toronto, ON, Canada, ³Toronto Rehabilitation Institute, University Health Network, Toronto, ON, Canada, ⁴Institute for Work & Health, Toronto, ON, Canada, ⁵University of Toronto, Krembil Neurosciences Centre, Toronto, ON, Canada, ⁶Department of Medicine, Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ⁷Brigham and Women's Hospital | Cumming School of Medicine, University of Calgary, Calgary, AB, Canada, ⁸University of California, San Francisco, Novato, CA, ⁹University of Toronto, Toronto, ON, Canada
Background/Purpose: Cognitive impairment (CI) is among the earliest and the most prevalent manifestations of SLE. Previous studies have demonstrated that the increased levels of interferon…
Abstract Number: 0472 • ACR Convergence 2020
ApoB:ApoA1 Ratio Could Predict Atherosclerotic Risk in Juvenile-SLE Patients Associated with Altered Interferon Signalling in CD8+ T-cells
George Robinson¹, Kirsty Waddington², Leda Coelewij², Junjie Peng², Meena Naja², Chris Wincup², Anna Radziszewska², Hannah Peckham², David Isenberg², Yiannis Ioannou², Ines Pineda Torra², Coziana Ciurtin² and Elizabeth Jury², ¹University College London, Hertford, United Kingdom, ²University College London, London, United Kingdom
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune rheumatic disease characterised by immune-dysregulation, chronic inflammation, type-I interferon (IFN) signatures and increased cardiovascular disease (CVD) risk…
Abstract Number: 1804 • ACR Convergence 2020
Impact of the Kynurenine/Tryptophan Pathway on Cognitive Dysfunction and Depression in Systemic Lupus Erythematosus
Erik Anderson¹, Joanna Fishbein², Joseph Hong², Julien Roeser³, Richard Furie⁴, Cynthia Aranow², Bruce Volpe², Betty Diamond⁵ and Meggan Mackay⁶, ¹Feinstein Institutes for Medical Research, New York, NY, ²Feinstein Institutes for Medical Research, Manhasset, NY, ³Charles River Laboratories, South San Francisco, CA, ⁴Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, ⁵Northwell Health, Manhasset, NY, ⁶Feinstein Institute for Medical Research, Manhasset, NY
Background/Purpose: Tryptophan (TRP) is metabolized to kynurenine (KYN), quinolonic acid [QA, a N-methyl D-aspartate receptor (NMDAR) agonist] and kynurenic acid (KA, an NMDAR antagonist). KYN/TRP…
Abstract Number: 0861 • ACR Convergence 2020
Hydroxychloroquine Use Is Associated with Diminished Type I Interferon-related Pathways in Systemic Lupus Erythematosus Patients
Samantha Slight-Webb¹, Kevin Thomas¹, Rufei Lu¹, Susan Macwana¹, Joan Merrill¹, Cristina Arriens¹, Eliza Chakravarty¹, Teresa Aberle¹, Holden Maecker², Paul Utz³, Joel Guthridge¹ and Judith James⁴, ¹Oklahoma Medical Research Foundation, Oklahoma City, OK, ²Institute for Immunity, Transplantation and Infection, Stanford University School of Medicine, Stanford, CA, ³Stanford University School of Medicine, Stanford, CA, ⁴Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation;Department of Pathology, University of Oklahoma Health Sciences Center;Department of Medicine, University of Oklahoma Health Sciences Center, Edmond, OK
Background/Purpose: Hydroxychloroquine (HCQ) has been used for decades to treat systemic lupus erythematosus (SLE) and is associated with decreased lupus flares and damage. However, despite…

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