Abstracts tagged "interferons"

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: 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: 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: 958 • 2019 ACR/ARP Annual Meeting
Identification of IFN-γ-producing Effector B Cells in Humans: Their Relevance to the Pathogenesis of Systemic Lupus Erythematosus
Kazuhiko Higashioka¹, Masahiro Ayano ², Yasutaka Kimoto ³, Hiroki Mitoma ², Mitsuteru Akahoshi ², Yojiro Arinobu ², Koichi Akashi ¹, Takahiko Horiuchi ⁴ and Hiroaki Niiro ⁵, ¹Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan, ²Department of Medicine and Biosystemic Science, Graduate School of Medical Sciences, Fukuoka, Japan, Fukuoka, Japan, ³Department of Internal Medicine, Kyushu University Beppu Hospital, Beppu, Japan, ⁴Department of Internal Medicine, Kyushu University Beppu Hospital, Beppu, Japan, Beppu, Japan, ⁵Clinical Education Centre, Kyushu University Hospital, Fukuoka, Japan, Fukuoka, Japan
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune, multi-systemic disease that potentially affects any organ in the body. Clinical efficacy of B cell-targeting therapy underscores…
Abstract Number: 2900 • 2019 ACR/ARP Annual Meeting
Interferon Signature Predicts Response to Tofacitinib in Haploinsufficiency of A20
Sarah Blackstone¹, Daniella Schwartz ¹, Natalia Sampaio Moura ¹, Deborah Stone ¹, Meryl Waldman ¹, Patrycja Hoffmann ¹, Anne Jones ¹, Tina Romeo ¹, Karyl Barron ¹, Joshua Milner ¹, Daniel Kastner ¹ and Amanda Ombrello ¹, ¹National Institutes of Health, Bethesda, MD
Background/Purpose: The protein A20, encoded by TNFAIP3, represses signaling upstream of the inflammatory transcription factor nuclear factor (NF)-kB by regulating ubiquitination. Heterozygous loss-of-function mutations in…
Abstract Number: 1026 • 2019 ACR/ARP Annual Meeting
Interferon Lambda Promotes Age-Associated B Cells
Jennifer Barnas¹, Nida Meednu ¹, Andrew McDavid ¹, Jennifer Albrecht ¹, Christopher Richardson ¹, R. John Looney ² and Jennifer Anolik ¹, ¹University of Rochester Medical Center, Rochester, NY, ²University of Rochester Medical Center, Rochester
Background/Purpose: Age-associated B cells (ABC), defined as CD11c+ T-bet+ or CD11c+CD21-, represent a subset of B lymphocytes that are increased in systemic lupus erythematosus (SLE)…
Abstract Number: 2909 • 2019 ACR/ARP Annual Meeting
Efficacy and Safety of the Selective Interleukin-1 Receptor Associated Kinase 4 Inhibitor, PF-06650833, in Patients with Active Rheumatoid Arthritis and Inadequate Response to Methotrexate
Spencer I Danto¹, Negin Shojaee ¹, Ravi Shankar P Singh ¹, Zorayr Manukyan ², Jessica Mancuso ¹, Elena Peeva ¹, Michael Vincent ¹ and Jean Beebe ¹, ¹Pfizer Worldwide Research & Development, Immunology, and Inflammation Research Unit, Cambridge, MA, ²Pfizer Worldwide Research & Development, Immunology, and Inflammation Research Unit, ..
Background/Purpose: Adaptive and innate immune pathways are involved in inflammation and pathogenesis of rheumatoid arthritis (RA). Toll-like receptor (TLR) stimulation activates the innate immune system,…
Abstract Number: 1296 • 2019 ACR/ARP Annual Meeting
Increased MxA Protein Expression and Dendritic Cells in Spongiotic Dermatitis Differentiates Dermatomyositis from Eczema
Majid Zeidi¹, Kristen Chen ¹, Basil Patel ², Rachel Lim 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, ³Corporal Michael J. Crescenz VAMC, Philadelphia, PA, USA, Philadelphia
Background/Purpose: Dermatomyositis (DM) is conventionally characterized by interface dermatitis (ID) on skin histopathology. A subset of patients with clinically diagnosed DM have skin biopsies showing…
Abstract Number: 1455 • 2019 ACR/ARP Annual Meeting
B and T Cell Immunologic Features Associated with Higher Disease Activity Score and Focus Score in Primary Sjögren Syndrome
Alice Mai¹, Yuriy Baglaenko ², Dario Ferri ³, Kieran Manion ⁴, Dennisse Bonilla ⁵, Arthur Bookman ⁶ and Joan Wither ⁷, ¹University of British Columbia Department of Rheumatology, Vancouver, Canada, ²Brigham Woman's Hospital, Boston, ³University Health Network, Toronto, ON, Canada, ⁴Krembil Research Institute, Toronto, ON, Canada, ⁵University Health Network, University of Toronto, Toronto, ON, Canada, ⁶University Health Network, Toronto, Canada, ⁷University Health Network, Krembil Research Institute, Toronto, ON, Canada
Background/Purpose: Primary Sjögren syndrome (pSS) is a systemic autoimmune disease with an incompletely understood pathogenesis and heterogenous disease manifestations. Identifying cellular immune changes that may…
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…

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