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: 2739 • 2019 ACR/ARP Annual Meeting
Skin Disease Activity and Autoantibody Phenotype Are Major Determinants of Blood Interferon Signatures in Dermatomyositis
Mika Tabata¹, Kavita Sarin ², Karen Page ³, Christine Huard ³, Shanrong Zhao ³, Donald Bennett ³, Jillian Johnson ³, Kristen Johnson ³ and David Fiorentino ², ¹Stanford University School of Medicine, Stanford, CA, ²Department of Dermatology, Stanford University School of Medicine, Redwood City, CA, ³Pfizer, Boston
Background/Purpose: Interferon (IFN) signaling is upregulated in dermatomyositis (DM) and thought to play a role in pathogenesis. An IFN gene signature in peripheral blood of…
Abstract Number: 78 • 2019 ACR/ARP Annual Meeting
Ultraviolet Light Induces Increased T Cell Activation in Lupus-Prone Mice via Type I Interferon-Dependent Inhibition of T Regulatory Cells
Sonya Wolf-Fortune ¹, Shannon Estadt ¹, Jonathan Theros ¹, Tyson Moore ¹, Jason Ellis ¹, Jianhua Liu ¹, Tamra Reed ¹, Chaim Jacob ¹, Johann Gudjonsson ¹ and J Michelle Kahlenberg¹, ¹University of Michigan, Ann Arbor, MI
Background/Purpose: Ultraviolet (UV) light is a known trigger of skin and possibly systemic inflammation in systemic lupus erythematosus (SLE) patients. Although type I interferons (IFN)…
Abstract Number: 2808 • 2019 ACR/ARP Annual Meeting
Linking Toll-Like Receptor Signaling and Type I Interferons to Inflammation and Fibrosis in a Macrophage/Fibroblast Model of Congenital Heart Block
Miao Chang¹, Robert Clancy ¹ and Jill Buyon ¹, ¹NYU School of Medicine, New York
Background/Purpose: Since one of the strongest associations with antibodies (abs) to SSA/Ro (Ro60) is the development of congenital heart block (CHB), this model provides an…
Abstract Number: 88 • 2019 ACR/ARP Annual Meeting
Type I Interferon Signature Activation in Antiphospholipid Syndrome: Gene Expression Heterogeneity Among Disease Subsets
Irene Cecchi¹, Massimo Radin ¹, Elena Rubini ¹, Silvia Grazietta Foddai ¹, Ana Suarez ², Elisa Menegatti ¹, Dario Roccatello ¹, Savino Sciascia ³ and Javier Rodriguez Carrio ², ¹University of Turin, Turin, Italy, ²University of Oviedo, Oviedo, Spain, ³Center of Research of Immunopathology and Rare Diseases- Coordinating Center of Piemonte and Valle d’Aosta Network for Rare Diseases, Department of Clinical and Biological Sciences, University of Turin, Torino, Italy
Background/Purpose: Type I Interferons (IFN) play a key role in the pathogenesis and evolution of various autoimmune diseases. Previous studies have demonstrated that the expression…
Abstract Number: 2813 • 2019 ACR/ARP Annual Meeting
Pentameric, but Not Monomeric C-reactive Protein, Limits the SnRNP-immune Complex Triggered Type I Interferon Response: Implications for Lupus Pathogenesis
Cecilia Svanberg ¹, Helena Enocsson ¹, Klara Martinsson ¹, Lawrence Potempa ², Ibraheem Rajab ², Jonas Wetterö ¹, Marie Larsson ¹ and Christopher Sjöwall³, ¹Linköping University, Linköping, Sweden, ²Roosevelt University, Chicago, IL, ³Department of Clinical and Experimental Medicine, Rheumatology/Division of Neuro and Inflammation Sciences, Linköping University, Linköping, Sweden
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune systemic disease affecting multiple organs and which is characterized by autoantibodies directed against nuclear constituents. Common autoantibody…
Abstract Number: 788 • 2019 ACR/ARP Annual Meeting
Type I Interferon Score and Interferon Induced Mediators CXCL10 and Neopterin Are Correlated with Disease Activity in Juvenile Dermatomyositis
Rebecca Nicolai¹, Ivan Caiello ², Rava' Lucilla ³, Silvia Rosina ⁴, Francesco Licciardi ⁵, Luisa Bracci Laudiero ², Angelo Ravelli ⁶, Fabrizio De Benedetti ⁷ and Gian Marco Moneta ², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Lazio, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ³Clinical Epidemiology Unit, Ospedale Pediatrico Bambino Gesù, Roma, Italy, Rome, Italy, ⁴Giannina Gaslini Institute, IRCCS Clinica Pediatrica e Reumatologia, Genoa, Italy, Genoa, Italy, ⁵Division of Pediatric Immunology and Rheumatology, Regina Margherita Children Hospital,Turin, Italy, Turin, Italy, ⁶IRCCS Istituto Giannina Gaslini, Università degli Studi di Genova, Genova, Italy, ⁷Bambino Gesù Children’s Hospital, Rome, Italy
Background/Purpose: Interferons (IFNs) seem to play an important role in the pathogenesis of juvenile dermatomyositis (JDM). Our group previously reported that expression of both type…
Abstract Number: 2898 • 2019 ACR/ARP Annual Meeting
Evaluation of the Transcriptome of Non-Lesional, Non-Sun Exposed Skin in Patients with Lupus Nephritis
Hemant Suryawanshi¹, Robert Clancy ², Evan Der ³, Peter Izmirly ², H Michael Belmont ⁴, Chaim Putterman ⁵, Jill Buyon ² and Thomas Tuschl ¹, ¹Rockefeller Research Laboratories, New York, ²NYU School of Medicine, New York, ³Albert Einstein College of Medicine, New York, ⁴NYU Langone Health, New York, NY, ⁵Albert Einstein College of Medicine, New York, NY
Background/Purpose: The impact of renal injury in lupus nephritis (LN) is widespread with consequences to resident cells in other tissue beds, even non-lesional, non-sun exposed…
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…

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