Abstracts tagged "interferon"

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…
Abstract Number: 1740 • ACR Convergence 2021
Efficacy of Anifrolumab in Patients with SLE Previously Treated with Biologics: Post Hoc Analysis of Data from 2 Phase 3 Trials
Richard Furie¹, Eric Morand², Kenneth Kalunian³, Konstantina Psachoulia⁴, Emmanuelle Maho⁵, Catharina Lindholm⁶ and Raj Tummala⁴, ¹Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ²Monash University, Melbourne, Australia, ³University of California, La Jolla, CA, ⁴BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, ⁵BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁶BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden
Background/Purpose: In the phase 3 TULIP-1 and TULIP-2 trials, anifrolumab, a type I IFN receptor mAb, improved disease activity in patients with SLE.1,2 We investigated…
Abstract Number: 1273 • ACR Convergence 2021
A 12-week Aerobic Exercise Training Program in Women with Systemic Lupus Erythematosus (SLE) Improves Fatigue, Mitochondrial Dysfunction and Associated Interferon Gene Signature
Sarfaraz Hasni¹, Marquis Chapman², Rebekah Feng¹, Anam Ahmad¹, Sarthak Gupta³, Mohammad Naqi¹, Adam Munday¹, Shajia Lu¹, Massimo Gadina², Zerai Manna¹, Xiaobai Li⁴, Yinghui Shi¹, Kalyani Mishra-Thakur¹, Michael Davis⁵, Jun Chu³, Elaine Poncio⁶, Yenealem Temesgen-Oyelakin⁷, Jonathan Martinez¹, Zoe Morris¹, Isabel Ochoa⁶, Shuichiro Nakabo⁸, Bart Drinkard¹, Gayle McCrossin¹, Marybeth Stockman¹, Mariana Kaplan¹, Leorey Saligan¹, Randall Keyser⁹, Leighton Chan¹ and Lisa Chin¹, ¹National Institutes of Health, Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, ³National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ⁴Clinical Center, National Institutes of Health, Bethesda, MD, ⁵NIAMS, Bethesda, MD, ⁶NIH/NIAMS, Bethesda, MD, ⁷National Institutes of Health (NIH), Bethesda, MD, ⁸NIAMS/NIH, Bethesda, MD, ⁹George Mason University, Bethesda
Background/Purpose: Fatigue in SLE patients is ubiquitous and is reported as one of the most debilitating symptoms. Yet mechanisms underlying the pathophysiology of SLE-related fatigue…
Abstract Number: 1741 • ACR Convergence 2021
Anifrolumab Results in Favorable Responses Regardless of SLE Disease Duration: Post Hoc Analysis of Data from 2 Phase 3 Trials
Kenneth Kalunian¹, Maria Dall'Era², Richard Furie³, Eric Morand⁴, Konstantina Psachoulia⁵, Emmanuelle Maho⁶, Catharina Lindholm⁷ and Raj Tummala⁵, ¹University of California, La Jolla, CA, ²University of California San Francisco, San Francisco, CA, ³Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ⁴Monash University, Melbourne, Australia, ⁵BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, ⁶BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁷BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden
Background/Purpose: In 2 phase 3 trials, TULIP-1 and TULIP-2, anifrolumab, a type I IFN receptor mAb, improved disease activity in patients with SLE.1,2 Here, we…
Abstract Number: 1365 • ACR Convergence 2021
Release of High-Mobility Group Box-1 After an Raynaud’s Attack Potentially Leads to Fibroblast Activation and Interferon-γ Induced Protein-10 Production in Systemic Sclerosis
Isabella Atzeni¹, Yehya Al-Adwi², Berber Doornbos-van der Meer², Amel Eman Abdulle², Anniek Van Roon², Alja J. Stel², Harry Van Goor², Andries Smit², Johanna Westra² and Douwe J Mulder², ¹University of Groningen, University Medical Centre Groningen, Groningen, Groningen, Netherlands, ²University of Groningen, University Medical Centre Groningen, Groningen, Netherlands
Background/Purpose: Raynaud’s Phenomenon (RP) leading to repetitive ischaemia and reperfusion (IR) stress, is the first recognisable sign of systemic sclerosis (SSc). Although RP has been…
Abstract Number: 1742 • ACR Convergence 2021
Efficacy of Anifrolumab in Serological Subgroups of Patients with SLE Participating in 2 Phase 3 Trials
Ian N. Bruce¹, Ronald Van Vollenhoven², Yoshiya Tanaka³, Eric Morand⁴, Richard Furie⁵, Konstantina Psachoulia⁶, Emmanuelle Maho⁷, Catharina Lindholm⁸, Christi Kleoudis⁹ and Raj Tummala⁶, ¹University of Manchester and NIHR Manchester Biomedical Research Centre, Manchester University Hospitals NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom, ²Amsterdam Rheumatology and Immunology Center, Amsterdam, Netherlands, ³University of Occupational and Environmental Health, Kitakyushu, Japan, ⁴Monash University, Melbourne, Australia, ⁵Zucker School of Medicine at Hofstra/Northwell Health, Great Neck, NY, ⁶BioPharmaceuticals R&D, AstraZeneca, Gaithersburg, MD, ⁷BioPharmaceuticals R&D, AstraZeneca, Cambridge, United Kingdom, ⁸BioPharmaceuticals R&D, AstraZeneca, Gothenburg, Sweden, ⁹BioPharmaceuticals R&D, AstraZeneca, Durham, NC
Background/Purpose: In the TULIP-2 and TULIP-1 trials of patients with SLE, the type I IFN receptor mAb anifrolumab resulted in higher BILAG–based Composite Lupus Assessment…
Abstract Number: 1432 • ACR Convergence 2021
Modulation of Auto-Inflammation with a Novel Selective Cyclic GMP-AMP Synthase (cGAS) Inhibitor in a Trex1-/- Model
Kelly Pike¹, Alexandre Caron¹, Emilie Bérubé¹, Ramsay Beveridge¹, Marc-Oliver Boily¹, Jason Burch¹, Valerie Dumais¹, Nadine Fradet¹, Samuel Gaudreault¹, Daniel McKay¹, Marianne Raymond¹, Eleftheria Seliniotakis¹, Daniel Sietsema³, Alexander Skeldon¹, Miguel St.-Onge¹, Li Wang² and Michael Crackower², ¹Ventus Therapeutics, Montréal, QC, Canada, ²Ventus Therapeutics, Waltham, MA

Background/Purpose: The detection of viral nucleic acids (NA) elicits a transient type I interferon (IFN) response central to antiviral immunity. Chronic type I IFN responses…
Abstract Number: 1856 • ACR Convergence 2021
Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous SSc
Ranjitha Karanth¹, Giuseppina Abignano², Vishal Kakkar², Rebecca Ross², Christopher Denton³ and Francesco Del Galdo², ¹Leeds Institute of Rheumatic and Musculoskeletal Medicine, LTHT, Leeds, United Kingdom, ²University of Leeds, Leeds, United Kingdom, ³University College London Division of Medicine, Centre for Rheumatology and Connective Tissue Diseases, London, United Kingdom
Background/Purpose: Limited cutaneous systemic sclerosis (lcSSc) carries a highly variable prognosis and to date there are no stratification tools to predict clinical outcomes. Evidence suggests…

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