Abstracts tagged "interferon"

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…
Abstract Number: 0961 • ACR Convergence 2021
Proteomic Profiling of MIS-C Patients Reveals Heterogeneity Relating to Interferon Gamma Dysregulation and Vascular Endothelial Dysfunction
Caroline Diorio¹, Rawan Shraim¹, Laura Vella¹, Josephine Giles², Amy Baxter², Derek Oldridge², Scott Canna¹, Sarah Henrickson¹, Kevin Mcnerney¹, Frances Balamuth¹, Chakkapong Burudpakdee¹, Jessica Lee¹, Tomas Leng¹, Alvin Farrell¹, Michele Lambert¹, Kathleen Sullivan¹, John Wherry³, David Teachey¹, Hamid Bassiri¹ and Edward Behrens¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³UPenn, Philadelphia, PA
Background/Purpose: Multi-system Inflammatory Syndrome in Children (MIS-C) is a major complication of the Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) pandemic in pediatric patients. Children…
Abstract Number: 1643 • ACR Convergence 2021
Increased Incidence of Pediatric SLE and Other Interferon Activated Diseases During COVID-19 Pandemic
Xinyu Dou¹, David Kaelber² and Hulya Bukulmez¹, ¹MetroHealth Medical Center, Case Western Reserve, Cleveland, OH, ²The MetroHealth System Campus of Case Western Reserve University, Cleveland, OH
Background/Purpose: COVID-19, as a member of the Coronavirus family, has been described to trigger host immune response via type I interferon (IFN) signaling pathways with…
Abstract Number: 0996 • ACR Convergence 2021
The Stimulator of Interferon Genes (STING) Protects from Bone Loss Through Regulation of Tonic and Induced Type I Interferon Pathways
Susan MacLauchlan¹, Priyanka Kushwaha¹, Albert Tai², Jia (Sijia) Chen³, Catherine Manning¹, Katherine Fitzgerald⁴, Shruti Sharma² and Ellen Gravallese⁵, ¹Brigham and Women's Hospital, Boston, MA, ²Tufts University School of Medicine, Boston, MA, ³Brigham and Women's Hospital, Cambridge, MA, ⁴University of Massachusetts Medical School, Worcester, MA, ⁵Brigham and Women's Hospital, Harvard Medical School, Chestnut Hill, MA
Background/Purpose: The intracellular DNA sensing Stimulator of Interferon Genes (STING) pathway is critical for detection of viral and bacterial pathogen DNA. Hyperactivating mutations in this…
Abstract Number: 1733 • ACR Convergence 2021
Classification of Patients with Systemic Lupus Erythematosus Enrolled in 2 Phase 3 Trials by EULAR/ACR 2019 Criteria
Martin Aringer¹, Ian N. Bruce², Richard Furie³, Eric Morand⁴, Emmanuelle Maho⁵, Catharina Lindholm⁶ and Raj Tummala⁷, ¹University Medical Center and Faculty of Medicine Carl Gustav Carus, TU Dresden, Dresden, Germany, ²University of Manchester and NIHR Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom, ³Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ⁴Monash University, Melbourne, Australia, ⁵BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁶BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁷BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD
Background/Purpose: The TULIP-1 and TULIP-2 trials of anifrolumab, an anti–type I IFN receptor mAb, enrolled autoantibody-positive (ANA, anti-dsDNA, and/or anti-Smith [anti-Sm]) patients, who fulfilled the…
Abstract Number: 1011 • ACR Convergence 2021
Validation of Bioinformatics Pipeline to Detect NEMO-Deleted Exon 5 Autoinflammatory Syndrome (NEMO-NDAS) and Preliminary Clinical and Immunologic Characterization
Adriana Almeida de Jesus¹, Bin Lin², Eric Karlins³, Dana Kahle⁴, Andre Rastegar², Jacob Mitchell², Sofia Torreggiani², Farzana Bhuyan², Sara Alehashemi⁵, Kader Cetin Gedik⁶, Kat Uss², Chyi-Chia Lee⁷, Hyesun Kuehn⁸, Sergio Rosenzweig⁸, Katherine Calvo⁸, Magdalena Walkiewicz⁹, Justin Lack¹⁰, Eric Hanson¹¹, Amer Khojah¹², Eveline Wu¹³, Christiaan Scott¹⁴, Timothy Ronan Leahy¹⁵, Emma MacDermott¹⁵, Orla Kileen¹⁵, Thaschawee Arkachaisri¹⁶, Zoran Gucev¹⁷, Kathryn Cook¹⁸, Vafa Mammadova¹⁹, Gulnara Nasrullayeva¹⁹, Scott Canna²⁰, Douglas Kuhns²¹, Clifton Dalgard²², Timothy Moran²³, Andrew Oler³ and Raphaela Goldbach-Mansky²⁴, ¹TADS/NIAID/NIH, Silver Spring, MD, ²TADS/NIAID/NIH, Bethesda, MD, ³BCBB/NIAID/NIH, Bethesda, MD, ⁴National Institutes of Health, Chevy Chase, MD, ⁵TADS/NIAID/NIH, Clarksville, MD, ⁶Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ⁷NCI/NIH, Bethesda, MD, ⁸CC/DLM/NIH, Bethesda, MD, ⁹CSI/NIAID/NIH, Bethesda, MD, ¹⁰NCBR/NIAID/NIH, Bethesda, MD, ¹¹Indiana University School of Medicine, Indianapolis, IN, ¹²Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, ¹³UNC Chapel Hill, Chapel Hill, NC, ¹⁴Paediatric Rheumatology, University of Cape Town, Cape Town, South Africa, ¹⁵Children’s Health Ireland (CHI) at Crumlin, Dublin, Ireland, ¹⁶KK Women's and Children's Hospital, SingHealth, Singapore, Singapore, ¹⁷University Children's Hospital, Medical Faculty Skopje, Skopje, Macedonia, ¹⁸Akron Childrens Hospital, Copley, OH, ¹⁹Azerbaijan Medical University, Baku, Azerbaijan, ²⁰Children's Hospital of Philadelphia, Philadelphia, PA, ²¹Frederick National Laboratory for Cancer Research/NIH, Frederick, MD, ²²TAGC/USUHS, Bethesda, MD, ²³University of North Carolina School of Medicine, Chapel Hill, NC, ²⁴NIH/NIAID, Potomac, MD
Background/Purpose: Splice site variants in IKBKG that lead to exon 5 deletion cause NEMO-deleted exon 5 autoinflammatory syndrome (NEMO-NDAS). NEMO-NDAS clinically mimics the interferonopathy chronic…
Abstract Number: 1739 • ACR Convergence 2021
SLE Treatment History and Anifrolumab Efficacy by Baseline Standard Therapies in Patients with SLE from 2 Phase 3 Trials
Susan Manzi¹, Richard Furie², Eric Morand³, Yoshiya Tanaka⁴, Gabriel Abreu⁵, Catharina Lindholm⁵ and Raj Tummala⁶, ¹Allegheny Health Network, Pittsurgh, PA, ²Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ³Monash University, Melbourne, Australia, ⁴University of Occupational and Environmental Health, Kitakyushu, Japan, ⁵BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁶BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD
Background/Purpose: In the phase 3 TULIP-1 and TULIP-2 trials, anifrolumab, a type I IFN receptor mAb, improved disease activity versus placebo in patients with moderate…
Abstract Number: 1096 • ACR Convergence 2021
CSF-specific CD8 T Cell Clonal Expansion in Neurosarcoidosis
Michael Paley¹, Brandi Baker², Steven Dunham³, Nicole Linskey³, Elisha Roberson³, David Clifford³ and Wayne Yokoyama³, ¹Washington University in St. Louis, Olivette, MO, ²Washington University in St. Louis, St. Louis, MO, ³Washington University School of Medicine, St. Louis, MO
Background/Purpose: Neuroinflammation is a severe manifestation of the systemic inflammatory disorders. Sarcoidosis, which leads to neurologic disease in 5-10 % of cases, has traditionally been…

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