Abstracts tagged "interferon"

Abstract Number: 0893 • ACR Convergence 2023
Involvement of Type I Interferon-responsive Myeloid Cells in Renal Inflammation in a Lupus Mouse Model
Trine Jorgensen and Lindsey Han, Lerner Research Institute, Cleveland Clinic Foundation, Cleveland, OH
Background/Purpose: Systemic Lupus Erythematosus (SLE) is an autoimmune disease that can cause damage to multiple organs, including the kidneys in Lupus Nephritis (LN). Current treatments…
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: 0091 • ACR Convergence 2023
Impaired X-Chromosome Inactivation Maintenance in T Cells Is Associated with Features of Reduced Disease Severity in a Toll-Like Receptor 7-Driven Model of Systemic Autoimmunity
Nikhil Jiwrajka¹, Zowie Searcy², Claudia Lovell², Natalie Toothacre², Katherine Forsyth² and Montserrat Anguera², ¹Divison of Rheumatology, Department of Medicine, Hospital of the University of Pennsylvania, Phildelphia, PA, ²Department of Biomedical Sciences, University of Pennsylvania School of Veterinary Medicine, Philadelphia, PA
Background/Purpose: Many systemic autoimmune rheumatic diseases, including systemic lupus erythematosus (SLE), Sjögren's syndrome, and systemic sclerosis are highly female-biased. Although these diseases are more prevalent…
Abstract Number: 0894 • ACR Convergence 2023
The Cellular and Spatial Type I Interferon Response Following Skin Exposure to Ultraviolet Light
Jie An¹, Xizhang Sun¹, Rayan Najjar¹, Connie Zhao¹, Paul Kong², Stephanie Weaver², Amanda Koehne², Matt Fitzgibbon² and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Fred Hutchinson Cancer Center, Seattle, WA
Background/Purpose: SLE patients characteristically have a prominent type I interferon (IFN-I) signature in lesional and non-lesional skin. We recently demonstrated that, following a single exposure…
Abstract Number: 1708 • ACR Convergence 2023
In Cis SOCS1 Variants Illustrate the Precise Regulation of Interferon Signaling Needed to Prevent Autoimmunity
Yan Du¹, Evan Hsu², Kailey Brodeur³, Meng Liu⁴, Mindy Lo⁵, Craig Platt³ and Pui Lee⁵, ¹Harvard medical school, Boston, MA, ²Wesleyan University, Newton, MA, ³Boston Children's Hospital, Boston, MA, ⁴Southern Medical University, Boston, MA, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Systemic autoimmunity can be driven by monogenic or polygenic risk variants. We aimed to characterize the genetic basis of disease in a family with…
Abstract Number: 0093 • ACR Convergence 2023
Single Cell RNA-seq and Mass Cytometry Reveal a Cytotoxic CD8 Effector T Cell Population Associated with Interstitial Lung Disease in Systemic Sclerosis Patients
Ye Cao¹, Takanori Sasaki², Richard Ainsworth³, Kim Taylor⁴, Nunzio Bottini⁵, Mehreen Elahee⁶, Edy Kim⁷, Francesco Boin³ and Deepak Rao⁷, ¹Brigham and Women's Hospital, Boston, MA, ²Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ³Cedars-Sinai Medical Center, Los Angeles, CA, ⁴University of California San Francisco, San Francisco, CA, ⁵University of California, San Francisco, CA, ⁶University of Pittsburgh Medical Center, Pittsburgh, PA, ⁷Brigham and Women's Hospital, Boston, MA
Background/Purpose: Interstitial lung disease (ILD) is a major cause of morbidity and mortality in systemic sclerosis (SSc). We aimed to identify features of circulating immune…
Abstract Number: 0910 • ACR Convergence 2023
17β-estradiol and B-cell Intrinsic Type I Interferon Amplify Toll-like Receptor 7 Signaling Loop in B Cells of Female Lupus Prone BXD2 Mice
Kathryn Sullivan, Yong Wang, Winn Chatham, John Mountz and Hui-chen Hsu, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: While systemic lupus erythematosus (SLE) disproportionally affects women versus men, elevation in estradiol (E2) alone is not sufficient to promote the development of autoantibody…
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…

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