Abstracts tagged "interferon"

Abstract Number: 1618 • ACR Convergence 2021
Disease Flares in CANDLE/PRAAS with Dose Reductions of Baricitinib
Kader Cetin Gedik¹, Grace Materne², Ana Ortega-Villa³, Gina Montealegre Sanchez⁴, Adam Reinhardt⁵, Paul Brogan⁶, Yackov Berkun⁷, Sara Murias⁸, Maria Robles⁹, Susanne Schalm¹⁰, Adriana Almeida de Jesus¹¹ and Raphaela Goldbach-Mansky¹², ¹Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Diseases Section, NIAID/NIH, Bethesda, TN, ³Biostatistics Research Branch, Division of Clinical Research, NIAID/NIH, Bethesda, ⁴NIAID/NIH, Bethesda, MD, ⁵Boys Town National Research Hospital, Omaha, NE, ⁶UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁷Hadassah Hebrew University Medical Center, Jerusalem, Israel, ⁸Hospital Infantil La Paz, Madrid, Spain, ⁹Eskenazi Health Center, IndianaPolis, IN, ¹⁰Rheumatologie im Zentrum, Munich, Germany, ¹¹TADS/NIAID/NIH, Silver Spring, MD, ¹²NIH/NIAID, Potomac, MD
Background/Purpose: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures /proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to treatment with baricitinib but require higher exposure…
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: L10 • ACR Convergence 2020
Targeting Plasmacytoid Dendritic Cells Improves Cutaneous Lupus Erythematosus Skin Lesions and Reduces Type I Interferon Levels: Results of a Phase 1 Study of VIB7734
Victoria Werth¹, Jodi Karnell², William Rees², Nanette Mittereder³, Li Yan², Yanping Wu³, Jorn Drappa², Gabor Illei² and John Ratchford², ¹University of Pennsylvania, Philadelphia, PA, ²Viela Bio, Gaithersburg, MD, ³Viela Bio, Gaithersburg
Background/Purpose: Plasmacytoid dendritic cells (pDCs) secrete large amounts of type I interferon (IFN) and other cytokines upon activation. pDCs migrate to sites of active disease…
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…
Abstract Number: 1805 • ACR Convergence 2020
Longitudinal Analysis of IFN Status and Disease Characteristics in SLE
Melissa Northcott¹, Alberta Hoi², Rachel Koelmeyer³ and Eric Morand⁴, ¹Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Victoria, Australia, ²Monash University, Melbourne, Victoria, Australia, ³Monash University, Clayton, Victoria, Australia, ⁴Medicine, School of Clinical Sciences at Monash Health, Monash University, Melbourne, Australia
Background/Purpose: The type 1 interferon (IFN) cytokine family is key to the pathogenesis of SLE, evidenced by the expression of IFN stimulated genes (ISGs) in…
Abstract Number: 0035 • ACR Convergence 2020
Distinct Biological Pathways in Both Blood and Kidney Further Define Molecular Profiles Across Diverse Nephritides
Loqmane Seridi¹, Matteo Cesaroni¹, Qingxuan Song², Ashley Orillion¹, Frédéric Baribaud¹, Tatiana Ort¹, Sheng Gao², Tomas Parker³, James Chevalier³, Dan Levine³, Alan Perlman³ and Jarrat Jordan¹, ¹Janssen Research & Development, LLC, Spring House, PA, ²Janssen Research & Development, LLC, Spring House, PA, USA, Spring House, PA, ³The Rogosin Institute, New York Presbyterian Hospital-Weill Medical College of Cornell University New York, NY, USA., New York, NY
Background/Purpose: Approximately 40% of SLE patients will develop Lupus Nephritis (LN), of which 10-30 % will progress to end-stage renal disease. To further understand LN…
Abstract Number: 0935 • ACR Convergence 2020
Efficacy and Safety Results from a Phase 2, Randomized, Double-Blind Trial of BIIB059, an Anti-Blood Dendritic Cell Antigen 2 Antibody, in SLE
Richard Furie¹, Ronald van Vollenhoven², Kenneth Kalunian³, Sandra Navarra⁴, Juanita Romero-Díaz⁵, Victoria Werth⁶, Xiaobi Huang⁷, Hua Carroll⁸, Adam Meyers⁷, Cristina Musselli⁷, Catherine Barbey⁹ and Nathalie Franchimont⁷, ¹Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, ²Department of Rheumatology, Amsterdam Rheumatology and Immunology Center, Amsterdam, Netherlands, ³University of California San Diego, La Jolla, CA, ⁴University of Santo Tomas, Manila, Philippines, ⁵Instituto Nacional de Ciencias Medicas y Nutricion Salvador Zubiran, Mexico City, Mexico, ⁶University of Pennsylvania and the Michael J. Crescenz VA Medical Center, Philadelphia, PA, ⁷Biogen, Cambridge, MA, ⁸Biogen, Cambridge, ⁹Biogen, Baar, Switzerland
Background/Purpose: Type I interferons (IFN-I), inflammatory mediators principally produced by plasmacytoid dendritic cells (pDCs), components of the innate immune system, have been implicated in the…
Abstract Number: 1806 • ACR Convergence 2020
The Association of Interferon-α with Kynurenine/Tryptophan Pathway Activation in Systemic Lupus Erythematosus
Erik Anderson¹, Ying Jin², Sara Goodwin², Julien Roeser³, Richard Furie⁴, Cynthia Aranow⁵, Bruce Volpe⁵, Betty Diamond⁶ and Meggan Mackay⁷, ¹Feinstein Institutes for Medical Research, New York, NY, ²Cold Spring Harbor Laboratory, Cold Spring Harbor, NY, ³Charles River Laboratories, South San Francisco, CA, ⁴Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, ⁵Feinstein Institutes for Medical Research, Manhasset, NY, ⁶Northwell Health, Manhasset, NY, ⁷Feinstein Institute for Medical Research, Manhasset, NY
Background/Purpose: Type I IFN contributes to SLE pathogenesis and stimulates the kynurenine/tryptophan (KYN/TRP) pathway, producing elevated quinolinic acid (QA) levels relative to kynurenic acid (KA)…

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