Abstracts tagged "interferon"

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…
Abstract Number: 2003 • ACR Convergence 2022
Anti-rheumatic Treatment Modulates Expression of the Glycolytic Enzyme PFKFB3 in CD14+ Monocytes of Patients with Rheumatoid Arthritis, Which Contributes to Dissimilarities of the IFN Signature
Malin Erlandsson¹, Karin Andersson¹, Sofia Töyrä Silfverswärd², Rille pullerits² and Maria Bokarewa², ¹Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Goteborg, Sweden, ²Department of Rheumatology and Inflammation Research, Institute of Medicine, Sahlgrenska Academy, University of Gothenburg, Göteborg, Sweden
Background/Purpose: Combination of IFN-stimulated genes known as IFN signature, helps to predict disease activity and treatment response in several autoimmune diseases, including rheumatoid arthritis (RA).…
Abstract Number: 0559 • ACR Convergence 2022
JAK Inhibitor Withdrawal Causes a Transient Proinflammatory Signaling Cascade in Minor Salivary Gland Mesenchymal Stromal Cells
Ilya Gurevic, jacques Galipeau and Sara McCoy, University of Wisconsin, Madison, WI
Background/Purpose: Sjögren's Disease (SjD) has high glandular IFNg levels, associated with disease activity and lymphoma risk. We previously showed IFNg-stimulated minor salivary gland (SG)-mesenchymal stromal…
Abstract Number: 0984 • ACR Convergence 2022
Impact of Hydroxychloroquine on Interferon Pathway Expression of SLE Phenotypic Subsets in the Absence of Background Medications
Colin Mowery, Miles Smith, Kevin Thomas, Susan Macwana, Wade deJager, Stan Kamp, Cristina Arriens, Teresa Aberle, Judith James, Joel Guthridge and Joan Merrill, Oklahoma Medical Research Foundation, Oklahoma City, OK
Background/Purpose: Chronic activation of interferon (IFN) pathways is a key driver of immune dysregulation in SLE. Hydroxychloroquine (HCQ) inhibits toll-like receptors 7 and 9 in…
Abstract Number: 2043 • ACR Convergence 2022
What Does Isolated Anti-Ro52 Antibody Positivity Mean in Sjögren’s Syndrome?
Eléonore Bettacchioli¹, Alain Saraux², Alice Tison³, Divi CORNEC⁴, Maryvonne Dueymes¹, Marta Alarcon-Riquelme⁵ and Valerie Devauchelle⁶, ¹Immunology and Immunotherapy Laboratory, CHRU Brest, Brest, France, ²CHU Brest, Brest, France, ³Rheumatology Department, CHRU Brest, Brest, France, ⁴CHRU Brest, Brest, France, ⁵Center for Genomics and Oncological Research (GENYO), Granada, Spain, ⁶Université de Bretagne Occidentale, Brest, France
Background/Purpose: Primary Sjögren's syndrome (pSS) is a systemic autoimmune disease characterized by a triad of dryness, pain and fatigue in affected patients. Its diagnosis is…
Abstract Number: 0584 • ACR Convergence 2022
Non-Clinical Evaluations of Deucravacitinib and Janus Kinase Inhibitor Specificity in Inflammatory or Homeostatic Pathways
Brandon Johnson, Jennifer Koenitzer, Peter Schafer and Ian M. Catlett, Bristol Myers Squibb, Princeton, NJ
Background/Purpose: Deucravacitinib is a selective inhibitor of tyrosine kinase 2 (TYK2) that has shown clinical efficacy in psoriasis, PsA, and SLE. Deucravacitinib is an allosteric…
Abstract Number: 1065 • ACR Convergence 2022
Biosamples from VEDOSS Patients Show Pathological Signs of SSc: Opportunity for a Biololgical Diagnosis of Disease
Rebecca Ross¹, Emily Clarke¹, Will Merchant¹, Panji Mulipa¹, Natalia Riobo-DelGaldo¹ and Francesco Del Galdo², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: The VEDOSS study has recently indicated that more than 50% of patients affected by Raynaud’s phenomenon (RP) and specific SSc anti-nuclear antibodies (ANA) and/or…
Abstract Number: 2051 • ACR Convergence 2022
Targeting Janus Kinase Pathway in Sjogren’s Disease Corrects IFN-Driven Inflammation and Epithelial Dysfunction
Sarthak Gupta¹, Eiko Yamada², Hiroyuki Nakamura², Zohreh Khavandgar³, Daniel Martin², Mayank Tandon⁴, Ilias Alevizos⁵, Shyh-Ing Jang², Paola Perez², Kalie Dominick², Thomas Pranzatelli², Alan Baer⁶, john chiorini⁴ and Blake Warner³, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²NIDCR, Bethesda, MD, ³National Institutes of Health, Bethesda, MD, ⁴NIH, Bethesda, MD, ⁵Horizon Therapeutics, Gaithersburg, MD, ⁶Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: Many of the inflammatory cytokines implicated in Sjogren's Disease (SjD) pathogenesis, in particular Type I and II interferons (IFNs), signal through Janus kinases (JAK)…
Abstract Number: L20 • ACR Convergence 2021
Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)
Fabrizio De Benedetti¹, Alexei Grom², Paul Brogan³, Claudia Bracaglia¹, Manuela Pardeo¹, Giulia Marucci¹, Despina Eleftheriou³, Charalampia Papadopoulou³, Pierre Quartier⁴, Jordi Antón⁵, Rikke Frederiksen⁶, Veronica Asnaghi⁶ and Cristina De Min⁶, ¹Bambino Gesù Children's Hospital IRCCS, Rome, Italy, ²Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ³UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁴Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, ⁵Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, ⁶Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland
Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…

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