Abstracts tagged "interferons"

Abstract Number: 027 • 2020 Pediatric Rheumatology Symposium
Clinical Features and Outcomes in Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE) Syndrome: Before and After JAK-inhibition
Sofia Torreggiani¹, Pascal Pillet ², Fabiano de Oliveira Poswar ³, Anna Kozlova ⁴, Anna Shcherbina ⁴, Marietta De Guzman ⁵, Jacob Mitchell ⁶, Gina A. Montealegre Sanchez ⁷, Katherine Townsend ⁸, Kim Johnson ⁹, Adriana Almeida de Jesus ¹⁰ and Raphaela Goldbach-Mansky ¹¹, ¹National Institutes of Health, Bethesda, ²Bordeaux Cedex, France, ³Hospital de Clinicas de Porto Alegre, Porto Alegre, Brazil, ⁴Center for Pediatric Hematology, Oncology, Immunology, Moscow, Russia, ⁵Section of Rheumatology, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, ⁶NIAID, NIH, Bethesda, ⁷NIH/ NIAID, Rockville, ⁸NIH, ⁹NIH, NIAID, Bethesda, ¹⁰NIAID, NIH, Silver Spring, ¹¹NIH/NIAID, Potomac
Background/Purpose: Chronic Atypical Neutrophilic Dermatosis With Lipodystrophy And Elevated Temperature (CANDLE) Syndrome is an autoinflammatory interferonopathy caused by mutations in the genes encoding for components…
Abstract Number: 079 • 2020 Pediatric Rheumatology Symposium
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Arianna De Matteis ¹, Denise Pires Marafon ², Ivan Caiello ², Manuela Pardeo ², Giulia Marucci ², Emanuela Sacco ², Giusi Prencipe ², Fabrizio De Benedetti ² and Claudia Bracaglia², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Lazio, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) and Secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm. Prompt recognition and early treatment are essential…
Abstract Number: 080 • 2020 Pediatric Rheumatology Symposium
Therapeutic Interferon Gamma Neutralization with Emapalumab in Patients with NRLC4- and CDC42-Associated Diseases Characterized by Recurrent and Severe Hemophagocytic Lymphohistiocytosis
Claudia Bracaglia¹, Antonella Insalaco ¹, Giulia Marucci ¹, Manuela Pardeo ¹, Emanuela Sacco ¹, Virginia Messia ¹, Giusi Prencipe ¹, Ivan Caiello ¹, Sarka Fingerhutova ², Pavla Dolezalova ², Veronica Asnaghi ³, Maria Ballabio ³, Cristina de Min ³ and Fabrizio De Benedetti ¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, ²Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, ³Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: Interferon gamma (IFNγ) is a pivotal mediator of HLH. Emapalumab (a fully human anti-IFNγ monoclonal antibody) is efficacious in patients with primary HLH. In…
Abstract Number: 140 • 2020 Pediatric Rheumatology Symposium
Serum Biomarkers in a German Cohort of Patients with Systemic Juvenile Idiopathic Arthritis and Their Relationship to Response to Interleukin-1 Blockade
Claas Hinze¹, Tanja Hinze ², Helmut Wittkowski ¹, Christoph Kessel ¹, Sabrina Fuehner ¹ and Dirk Foell ¹, ¹University Hospital Muenster, Muenster, Nordrhein-Westfalen, Germany, ²Muenster, Nordrhein-Westfalen, Germany
Background/Purpose: Most, but not all, patients with systemic juvenile idiopathic arthritis (systemic JIA) respond to therapy with interleukin (IL)-1 blocking agents but predictive factors have…
Abstract Number: 168 • 2020 Pediatric Rheumatology Symposium
Examining the Role of IFN-I and Langerhans Cell ADAM17 in Lupus Photosensitivity
Keila Veiga¹, Noa Schwartz ², William Shipman ¹, Marvin Sandoval ³, Thomas Li ¹, Yurii Chinenov ¹, David Oliver ¹, Ali Jabbari ⁴, James Krueger ⁵, Sarah Taber ¹, Niroshana Anandasabapathy ⁶ and Theresa Lu ¹, ¹Hospital for Special Surgery, New York, ²Albert Einstein College of Medicine/Montefiore Medical Center, New York, ³Weill Cornell Graduate School of Medical Sciences, New York, ⁴University of Iowa, Iowa City, ⁵Rockefeller Center, New York, ⁶Weill Cornell Medicine, New York
Background/Purpose: Photosensitivity resulting in inflammatory skin lesions is a hallmark of cutaneous lupus. Lesions can be disfiguring and have a negative impact on quality of…
Abstract Number: 005 • 2020 Pediatric Rheumatology Symposium
Single Cell Sequencing of the Skin to Define Cell Populations of Interest in Localized Scleroderma (LS)
Emily Mirizio ¹, Wei Chen ², Tao Sun ², Tracy Tabib ³, Kaila Schollaert-Fitch ¹, Robert Lafyatis ⁴, Heidi Jacobe ⁵ and Kathryn Torok¹, ¹Pediatric Rheumatology, Univ of Pittsburgh Med Ctr, Pittsburgh, ²Research Computing Core at Children's Hospital of Pittsburgh, Pittsburgh, ³Univ of Pittsburgh Med Ctr, Pittsburgh, ⁴Univ of Pittsburgh Med Ctr, Pittsburgh, Pittsburgh, ⁵University of Texas Southwestern Medical Ctr, Dallas
Background/Purpose: Scleroderma is an autoimmune disorder involving inflammatory driven fibrosis, which encompasses systemic sclerosis (SSc) and localized scleroderma (LS). LS and SSc share histological characteristics,…
Abstract Number: L06 • 2019 ACR/ARP Annual Meeting
Interferon-gamma (IFN-γ) Neutralization with Emapalumab and Time to Response in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (s-JIA) who failed High-Dose Glucocorticoids
Fabrizio De Benedetti¹, Paul Brogan ², Alexei A. Grom ³, Pierre Quartier ⁴, Rayfel Schneider ⁵, Jordi Antón ⁶, Claudia Bracaglia ⁷, Manuela Pardeo ⁸, Giulia Marucci ⁸, Emanuela Sacco ⁹, Despina Eleftheriou ¹⁰, Charalampia Papadopoulou ¹¹, Philippe Jacqmin ¹², maria ballabio ¹³ and Cristina de Min ¹³, ¹Bambino Gesù Children's Hospital, Rome, Italy, ²Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, ³Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Necker Hospital, Paris, France, ⁵Hospital for Sick Children, Toronto, ON, Canada, ⁶Hospital Sant Joan de Déu, Barcelona, Spain, ⁷Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁸Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, ¹⁰UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section,, london, United Kingdom, ¹¹UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section, london, United Kingdom, ¹²MnS, Dinant, Belgium, ¹³Sobi AG, basel, Switzerland
Background/Purpose: MAS is a severe complication of rheumatic diseases, most frequently sJIA and adult-onset Still’s disease. It is characterized by fever, hepatosplenomegaly, liver dysfunction, cytopenias,…
Abstract Number: 1762 • 2019 ACR/ARP Annual Meeting
UV Light Induces Acute Type I Interferon Production in the Skin and Blood Which Is cGAS Dependent
Sladjana Skopelja-Gardner¹, Jie An ², Xizhang Sun ², Lena Tanaka ², Joyce Tai ², Payton Hermanson ², Masaoki Kawasumi ² and Keith Elkon ², ¹University of Washington, Seattle, WA, ²University of Washington, Seattle
Background/Purpose: Interferon response genes (ISG) are strongly expressed in the skin of many patients with systemic lupus erythematosus (SLE). Since plasmacytoid dendritic cells (pDC) are…
Abstract Number: 1866 • 2019 ACR/ARP Annual Meeting
Gene Expression Meta-Analysis Reveals Commonalities in Gene Activation and Enrichment of Immune Pathways and Cell Types in Dermatomyositis Target Tissues
Jessica Neely¹, Dmitry Rychkov ¹, Manish Paranjpe ¹, Michael Waterfield ², Susan Kim ¹ and Marina Sirota ², ¹University of California, San Francisco, San Francisco, CA, ²University of California, San Francisco, San Francisco
Background/Purpose: Dermatomyositis (DM) is a complex immune-mediated disease resulting in muscle and skin inflammation. Prior studies of gene expression in DM have revealed a type…
Abstract Number: 1869 • 2019 ACR/ARP Annual Meeting
Myeloid Dendritic Cells (mDCs) Are Major Producers of Interferon-β in Dermatomyositis and Higher Numbers of mDCs Are Found in Hydroxychloroquine Nonresponders
Kristen Chen¹, Majid Zeidi ¹, Maria Wysocka ², Nithin Reddy ¹, Arvin Jadoo ¹, Muhammad Bashir ¹, Sarah Ahmed ², Basil Patel ², Kevin Zhang ², Barbara White ³ and Victoria Werth ¹, ¹Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA and Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, PA, ²Department of Dermatology, University of Pennsylvania, Philadelphia, PA, USA, Philadelphia, PA, ³Corbus Pharmaceuticals, Norwood, MA
Background/Purpose: Dermatomyositis (DM) is an autoimmune disease affecting the skin, skeletal muscle, lungs, and/or other organs. While the pathogenesis remains poorly understood, it is thought…
Abstract Number: 1914 • 2019 ACR/ARP Annual Meeting
Increased Risk of Progression to Lupus Nephritis for Lupus Patients with Elevated Interferon Signature
Cristina Arriens¹, Quratul Raja ², Syed Ali Husain ², Bessy George ², Majid Abedi ³, Aviva Jacobs ⁴, Timothy Guyon ⁴, Hemani Wijesuriya ³, Teresa Aberle ⁵, Aikaterini Thanou ⁵, Stan Kamp ⁵, Susan R. Macwana ⁵, Eliza F. Chakravarty ¹, Joan T. Merrill ⁶, Judith James ¹, Robert Terbrueggen ⁴ and Joel Guthridge ¹, ¹Oklahoma Medical Research Foundation, University of Oklahoma Health Sciences Center, Oklahoma City, OK, ²University of Oklahoma Health Sciences Center, Oklahoma City, OK, ³DxTerity Diagnostics Inc, Anaheim, CA, ⁴DxTerity Diagnostics Inc, Rancho Dominguez, CA, ⁵Oklahoma Medical Research Foundation, Oklahoma City, OK, ⁶Okalahoma Medical Research Foundation, Oklahoma City, OK
Background/Purpose: The interferon (IFN) signature in SLE is well established, distinguishing lupus patients from healthy controls. Additionally, within lupus patients, higher levels of IFN-responsive gene…
Abstract Number: 2026 • 2019 ACR/ARP Annual Meeting
MicroRNA-27a Can Contribute to Interferon Signatures in Systemic Lupus Erythematosus via the Suppression of Tripartite Motif-containing Protein 27
Daiga Kishimoto¹, Ryusuke Yoshimi ¹, Yosuke Kunishita ¹, Yumiko Sugiyama ², Takaaki Komiya ¹, Natsuki Sakurai ³, Reikou Kamiyama ¹, Yohei Kirino ⁴ and Hideaki Nakajima ⁴, ¹Department of Stem Cell and Immune Regulation / Yokohama City University Graduate School of Medicine, Yokohama, Japan, ²Department of Rheumatic Diseases / Yokohama City University Medical Center, Yokohama, Japan, ³Department of Stem Cell and Immune Regulation / Yokohama City University Graduate School of Medicine, Yokohama City, Japan, ⁴Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine, Yokohama, Japan
Background/Purpose: Type Ⅰ interferons (IFN) contribute to antiviral innate immune responses. Upon viral infection, pattern recognition receptors trigger TANK-binding kinase 1 (TBK1) activation and lead…
Abstract Number: 2032 • 2019 ACR/ARP Annual Meeting
Enhanced IFN a Production and STING Pathway in Monocytes in Systemic Lupus Erythematosus Is Suppressed by the Inhibition of mTOR Activation
Goh Murayama¹, Asako Chiba ², Ayako Makiyama ³, Taiga Kuga ⁴, Ken Yamaji ⁴, Naoto Tamura ⁴ and Sachiko Miyake ², ¹Department of Internal Medicine and Rhumatology, Juntendo University School of Medicine, Tokyo, Japan, ²Department of Immunology, Juntendo University School of Medicine, Tokyo, Japan, ³Juntendo University School of Medicine, Tokyo, Japan, ⁴Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan
Background/Purpose: Interferona (IFNa) is increased and plays an important role in the pathogenesis of systemic lupus erythematosus (SLE). Overexpression of type I IFN regulated genes…
Abstract Number: 2033 • 2019 ACR/ARP Annual Meeting
IRAK4 Inhibition Suppresses TLR7, TLR9, and SLE Serum-Induced IFNA Production in Primary Human Plasmacytoid Dendritic Cells
Angie Hammond¹, Sean Parghi ¹, Nathan Wright ¹, Ethan Grant ¹, James Taylor ¹ and Matthew Warr ¹, ¹Gilead Sciences, Inc., Foster City, CA
Background/Purpose: A hallmark of lupus is the presence of antinuclear autoantibodies, including those against RNA-protein complexes and double-stranded DNA (dsDNA).1,2 FcγR-mediated internalization of these nucleic…
Abstract Number: 2563 • 2019 ACR/ARP Annual Meeting
PK/PD, Safety and Exploratory Efficacy of Subcutaneous Anifrolumab in SLE: A Phase-II Study in Interferon Type I High Patients with Active Skin Disease
Ian Bruce¹, Alireza Nami ², Erik Schwetje ³, M Edward Pierson ⁴, Yen Lin Chia ⁵, Denison Kuruvilla ⁵, Gabriel Abreu ⁶, Raj Tummala ³ and Catharina Lindholm ⁶, ¹University of Manchester, Manchester, United Kingdom, Manchester, England, United Kingdom, ²Joint & Muscle Medical Care, Charlotte, NC, US, Charlotte, NC, ³AstraZeneca, Gaithersburg, MD, USA, Gaithersburg, MD, ⁴AstraZeneca, Gaithersburg, MD, US, Gaithersburg, MD, ⁵AstraZeneca, South San Francisco, CA, US, South San Francisco, CA, ⁶AstraZeneca, Gothenburg, Sweden, Gothenburg, Sweden
Background/Purpose: Anifrolumab, a fully human anti–IFN Type I receptor mAb, is under investigation for the treatment of SLE at a dose of 300 mg intravenously…

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