Abstracts tagged "interferon"

Abstract Number: 1665 • ACR Convergence 2023
Endothelial Response to Type I Interferon Contributes to Vasculopathy and Fibrosis and Predicts Disease Progression of Systemic Sclerosis
Hanlin Yin¹, Oliver Distler², Bin Li³, Qingran Yan⁴ and Liangjing Lu¹, ¹Shanghai Jiao Tong University School of Medicine Affiliated Renji Hospital, Shanghai, China, ²Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ³Shanghai institute of immunology, Shanghai, China, ⁴Department of Rheumatology, Renji Hospital, Shanghai Jiaotong Univeisty School of medcine, Shanghai, China
Background/Purpose: Type I interferon (IFN-1) signature is a hallmark of patients with systemic sclerosis (SSc). However, its significance in clinical stratification and contribution to deterioration…
Abstract Number: 023 • 2023 Pediatric Rheumatology Symposium
Effect of Type 1 Interferons and JAK Inhibitors on Gene Expression in Bioengineered Pediatric Skeletal Muscle
Lauren Covert¹, Joseph Prinz², Hailee Patel³, Jeffrey Dvergsten⁴ and George Truskey³, ¹Duke University, Durham, NC, ²Duke University School of Medicine, Department of Biostatistics and Bioinformatics, Durham, NC, ³Duke University, Department of Biomedical Engineering, Durham, NC, ⁴Duke University Hospital, Durham, NC
Background/Purpose: Genetic studies of new-onset juvenile dermatomyositis (JDM) exhibit elevation of Type 1 interferons (IFN 1) IFNα and IFNβ in blood, skin, and muscle. To…
Abstract Number: 075 • 2023 Pediatric Rheumatology Symposium
Emapalumab Treatment Followed by Hematopoietic Stem Cell Transplantation in Systemic Juvenile Idiopathic Arthritis Complicated by Recurrent Macrophage Activation Syndrome
Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci², Simona Riccio², Francesco Quagliarella³, Ivan Caiello², Giusi Prencipe², Pietro Merli³, Franco Locatelli³ and Fabrizio De Benedetti¹, ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, ³Ospedale Pediatrico Bambino Gesù, Department of Hematology/Oncology, Cell and Gene Therapy, Roma, Italy
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of different rheumatic diseases, particularly of systemic juvenile idiopathic arthritis (sJIA).Methods: We report the case of…
Abstract Number: 080 • 2023 Pediatric Rheumatology Symposium
Analysis of Proteasomal Activity – a Potential Diagnostic Tool for Proteasome-associated Autoinflammatory Syndromes (PRAAS)
Yoel Levinsky¹, Oded Scheuerman², Rotem Tal³, Gil Amarilyo³ and Liora Harel³, ¹Schneider Children's Medical Center of Israel, Tel Aviv University, Petach Tikva, Israel, ²Pediatric B department, Schneider children's medical center of Israel, Petach Tikva, Israel, ³Pediatric rheumatology clinic, Schneider children's medical center of Israel, Petach Tikva, Israel
Background/Purpose: Interferonopathies are a recently recognized group of genetic syndromes associated with uncontrolled activation of interferon. PRAAS (proteasome-associated autoinflammatory syndromes) is an interferonopathy caused by…
Abstract Number: 0359 • ACR Convergence 2022
Attainment of Remission with Anifrolumab: A Post Hoc Analysis of Pooled TULIP-1 and TULIP-2 Datasets
Ronald Van Vollenhoven¹, Eric F. Morand², Richard A. Furie³, Ian N. Bruce⁴, Gabriel Abreu⁵, Raj Tummala⁶, Hussein Al-Mossawi⁷ and Catharina Lindholm⁸, ¹Department of Rheumatology, Amsterdam Rheumatology and Immunology Center, University of Amsterdam, Amsterdam, Netherlands, ²Monash University, Melbourne, Australia, ³Northwell Health, Great Neck, NY, ⁴The University of Manchester, Manchester, United Kingdom, ⁵AstraZeneca, Mölndal, Sweden, ⁶AstraZeneca, Gaithersburg, MD, ⁷AstraZeneca, Cambridge, United Kingdom, ⁸AstraZeneca, Gothenburg, Sweden
Background/Purpose: In patients with SLE, achieving remission is a treat-to-target goal. Remission is associated with lower rates of hospitalization and damage accrual and better quality…
Abstract Number: 0669 • ACR Convergence 2022
Understanding How Type I Interferon Modulates Langerhans Cell ADAM17 to Promote Photosensitivity in Lupus
Thomas Li¹, Ethan Seltzer¹, Victoria Zyulina¹, Keila Veiga², Noa Schwartz³, Yurii Chinenov¹, David Oliver¹, Jose Lora⁴, Ali Jabbari⁵, Yong Liu¹, Hafsa Munir¹, William Shipman¹, Marvin Sandoval¹, Isabel Sollohub¹, william ambler¹, Bikash Mishra⁴, sarah taber¹, karen onel¹, Mehdi Rashighi⁶, james krueger⁷, Niroshana Anandasabapathy⁴, Carl Blobel¹ and theresa Lu¹, ¹Hospital for Special Surgery, New York, NY, ²Maria Fareri Children's Hospital, New York, NY, ³Albert Einstein College of Medicine, New York, NY, ⁴Weill Cornell Medicine, New York, NY, ⁵University of Iowa, Iowa City, IA, ⁶University of Massachusetts Medical School, Worcester, MA, ⁷Rockefeller University, New York, NY
Background/Purpose: Photosensitivity is a hallmark symptom of lupus erythematosus (LE), in which patients develop inflammatory skin lesions in response to ultraviolet radiation (UVR). In examining…
Abstract Number: 1879 • ACR Convergence 2022
Analysis of the Association Between the Atrophic Factors Tripartite Motif Containing (TRIM) 63 and Atrogin-1 and the Clinical and Inflammatory Features of Patients with Idiopathic Inflammatory Myopathies
jiram torres-Ruiz¹, Abdiel Absalón-Aguilar², Juan Alberto Reyes-Islas², Alfredo Pérez-Fragoso², Nancy R Mejía-Domínguez³, guillermo Juárez-Vega⁴, Alejandro Alfaro-Goldaracena⁵, Beatriz Alcalá-Carmona², Guillermo Juárez-Vega³, Fabiola Cassiano-Quezada² and Diana Gómez-Martín¹, ¹INCMNSZ, Ciudad de México, Mexico, ²Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, Mexico, ³Red de Apoyo a la Investigación, Universidad Nacional Autónoma de México, Ciudad de México, Mexico, ⁴Red de Apoyo a la Investigación. UNAM, Ciudad de México, Mexico, ⁵Department of Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, Mexico
Background/Purpose: Muscle atrophy is mediated by the ubiquitination of myofilaments by two ubiquitin ligases called Tripartite Motif Containing (TRIM) 63 and Atrogin-1, which are induced…
Abstract Number: 0360 • ACR Convergence 2022
Longitudinal Variation of Proteomic Biomarkers That Correlate with Efficacy Endpoints: Results from a Phase 3 Trial of Anifrolumab in Moderate to Severe Systemic Lupus Erythematosus
Mark Lazarus¹, Paul Newcombe¹, Richard A. Furie², Philip Brohawn³, Wendy White³, Dominic Sinibaldi³, Nicola Ferrari¹, Raj Tummala³, Hussein Al-Mossawi¹, Edward Vital⁴, Eric F. Morand⁵, Daniel Muthas⁶ and Madhu Ramaswamy³, ¹AstraZeneca, Cambridge, United Kingdom, ²Northwell Health, Great Neck, NY, ³AstraZeneca, Gaithersburg, MD, ⁴University of Leeds, Leeds, England, United Kingdom, ⁵Monash University, Melbourne, Australia, ⁶AstraZeneca, Gothenburg, Sweden
Background/Purpose: Phase 2/3 clinical trials in patients with moderate to severe SLE have demonstrated that anifrolumab, a monoclonal antibody blocking IFNAR1, produced better clinical outcomes…
Abstract Number: 0740 • ACR Convergence 2022
Real-World Demographics, Clinical Characteristics, and Treatment Patterns of Patients Treated with Emapalumab for Secondary Hemophagocytic Lymphohistiocytosis in the United States: The REAL-HLH Study
Carl Allen¹, Shanmuganathan Chandrakasan², Michael Jordan³, Jennifer Leiding⁴, Abiola Oladapo⁵, Priti Pednekar⁶, Kelly Walkovich⁷ and John Yee⁸, ¹Baylor College of Medicine, Houston, TX, ²Emory University School of Medicine, Atlanta, GA, ³University of Cincinnati, Cincinnati, OH, ⁴Johns Hopkins University, Baltimore, MD, ⁵Swedish Orphan Biovitrum AB, Boston, MA, ⁶PRECISIONheor, Los Angeles, CA, ⁷University of Michigan Health, Ann Arbor, MI, ⁸Sobi - North America, Waltham, MA
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome caused by overproduction of proinflammatory cytokines, e.g., interferon gamma (IFNγ). Secondary HLH (sHLH), a subtype…
Abstract Number: 1931 • ACR Convergence 2022
NOD2 Mutations Are Associated with Upregulated Type 1 Interferon Gene Expression and Development of Granulomatous Hepatitis in Children with Autoimmune Hepatitis
Esraa Eloseily¹, Alexander Valencia², Astha Malik², Rebekah Karns², Cyd Castro Rojas², Mosab Alquraish², Rebecca Marsh³, Alexei Grom⁴ and Alexander Miethke², ¹Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Division of Bone Marrow Transplantation & Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Mutations in the gene NOD2 encoding the nucleotide-binding oligomerization domain-containing protein 2 (NOD2) which controls innate responses to LPS have been linked to Blau…
Abstract Number: 0361 • ACR Convergence 2022
Gene Expression Pathways Modulated by Anifrolumab in Systemic Lupus Erythematosus
Tina Baker¹, Sharifian Hoda², Paul Newcombe¹, Mark Lazarus¹, Madhu Ramaswamy³, Nicola Ferrari¹, Daniel Muthas², Hussein Al-Mossawi¹, Raj Tummala³, Eric F. Morand⁴, Richard A. Furie⁵, Edward Vital⁶ and Philip Brohawn³, ¹AstraZeneca, Cambridge, United Kingdom, ²AstraZeneca, Gothenburg, Sweden, ³AstraZeneca, Gaithersburg, MD, ⁴Monash University, Melbourne, Australia, ⁵Northwell Health, Great Neck, NY, ⁶University of Leeds, Leeds, England, United Kingdom
Background/Purpose: Anifrolumab is a monoclonal antibody to IFN-α receptor 1 that inhibits type I IFN signaling. In the phase 3 TULIP-1 and TULIP-2 trials, anifrolumab…
Abstract Number: 0974 • ACR Convergence 2022
Rapid Efficacy of Anifrolumab Across Multiple Subtypes of Recalcitrant Cutaneous Lupus Erythematosus Parallels Discrete Changes in Transcriptomic and Cellular Biomarkers
Lucy Marie Carter¹, Zoe Wigston¹, Jack Arnold¹ and Philip Laws², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Teaching Hospitals NHS Trust, Leeds, United Kingdom
Background/Purpose: Cutaneous lupus eyrthematosus (CLE) is frequently refractory to immunosuppressive therapies including B-cell depletion, but response varies by morphology with the chronic discoid (DLE) subtype…
Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Sara Alehashemi¹, Adriana Almeida de Jesus², Jonas Johannes Papendorf³, Farzana Bhuyan⁴, Kat Uss⁵, Anvitha Metpally⁶, Bin Lin⁷, Sophia Park⁸, Thais C.L. Moura⁹, Renata R Dias⁹, Mayra B. Dorna⁹, Katia Kozu⁹, Adriana Sallum⁹, Lucia M. A. Campos⁹, Gijs Santen¹⁰, Jesper Kers¹⁰, Onno Teng¹¹, Karin Palmblad¹², Tom Huizinga¹⁰, Frédéric Ebstein¹³, Elke Krüger¹³ and Raphaela Goldbach-Mansky¹⁴, ¹NIH/NIAID/TADS, Clarksville, MD, ²NIAID, NIH, Silver Spring, MD, ³Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Greifswald, Germany, ⁴National Institutes of Health, Bethesda, MD, ⁵National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁶NIAID, NIH, Bethesda, MD, ⁷NIH, Bethesda, MD, ⁸NIAID, Bethesda, MD, ⁹Children's Hospital of the University of São Paulo, São Paulo, Brazil, ¹⁰Leiden University Medical Center, Leiden, Netherlands, ¹¹Leiden University Medical Center, Leiderdorp, Netherlands, ¹²Karolinska University Hospital, Stockholm, Sweden, ¹³Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ¹⁴NIH/NIAID, Potomac, MD
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…
Abstract Number: 0556 • ACR Convergence 2022
Immune Checkpoint VISTA Regulates Type I Interferon (IFN-I) Production and Controls UV Light Triggered Skin IFN-I Response
Zachary Peters¹, Lindsay Mendyka¹, Sicong Shan¹, Angelique Cortez¹, William Rigby², Christopher Burns¹, Randolph Noelle³ and Sladjana Skopelja-Gardner¹, ¹Dartmouth Hitchcock Medical Center, Lebanon, NH, ²Dartmouth Hitchcock Medical Center, Lebanon, PA, ³Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: Most lupus patients chronically exhibit higher IFN-I scores in the skin, peripheral blood, and kidneys that is exacerbated by ultraviolet (UV) light. In normal…
Abstract Number: 0975 • ACR Convergence 2022
Modulation of T, B, and Innate Cell-Associated Pharmacodynamic Biomarkers in a Phase 3 Trial of Anifrolumab in Moderate to Severe SLE
Paul Newcombe¹, Richard A. Furie², Yoshiya Tanaka³, Wendy White⁴, Dominic Sinibaldi⁴, Philip Brohawn⁴, Mark Lazarus¹, Nicola Ferrari¹, Raj Tummala⁴, Hussein Al-Mossawi¹, Andre Nogueria da Costa⁵, Daniel Muthas⁵ and Madhu Ramaswamy⁴, ¹AstraZeneca, Cambridge, United Kingdom, ²Northwell Health, Great Neck, NY, ³University of Occupational and Environmental Health, Kitakyusyu Fukuoka, Japan, ⁴AstraZeneca, Gaithersburg, MD, ⁵AstraZeneca, Gothenburg, Sweden
Background/Purpose: SLE has been associated with expression of type I IFN gene signatures (IFNGS).1 Anifrolumab, a monoclonal antibody binding IFN receptor subunit 1, inhibits downstream…

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