Abstracts tagged "interferon"

Abstract Number: 1922 • ACR Convergence 2023
Use of Plasma with High Titer Neutralizing Autoantibodies to Type I Interferons in Patients with Severe Refractory Flare-up of Hidradenitis Suppurativa as Novel Passive Immunotherapy Approach: Trial Protocol
Charline Vauchy¹, Maxime Desmarets¹, Paul Bastard², Anne Puel², Pascale Richard³, Jean Laurent Casanova², Pierre Tiberghien³, Eric Toussirot⁴ and Francois Aubin⁵, ¹INSERM CIC-1431, Besançon, France, ²IHU Imagine, AP-HP Necker, Paris, France, ³Etablissement Français du Sang, St. Denis, France, ⁴University Hospital of Besancon, Besançon, France, ⁵Dermatology, University Hospital of Besancon, Besançon, France
Background/Purpose: Hidradenitis suppurativa (HS) is a multifactorial auto-inflammatory disorder with a prevalence of 1% in North American and European populations. HS may be associated with…
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: 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…

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