Abstracts tagged "interferon"

Abstract Number: 2329 • ACR Convergence 2023
AMTX-100, a Nuclear Transport Inhibitor, Attenuates Inflammatory Cytokine Production in vitro and Following UV Mediated Skin Inflammation in a Mouse Model of Cutaneous Lupus Erythematosus in vivo
Xizhang Sun¹, Jie An¹, Ting Wang¹, Arpit Rathee¹, Vernon Alvarez², Matthew Gonda³, Christian Lood¹ and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Amytrx Therapeutics Inc., Nashville, TN, ³Amytrx Therapeutics, Nashville, TN
Background/Purpose: Inflammatory stimuli induce transcription factors (TFs) such as NF-kB and interferon regulatory factors (IRFs). TFs are transported from cytosol to nucleus to activate genes…
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: 0635 • ACR Convergence 2022
Blood RNA-sequencing Reveals Complex Immune Dysregulation in ANA-positive Individuals with Distinct Profiles Preceding Progression to SLE versus Stable Autoimmunity
Lucy Marie Carter¹, Md Yuzaiful Md Yusof², Darren Plant³, Julien Bauer⁴, Stephanie Wenlock⁴, Adewonuola Alase¹, Antonios Psarras¹, Zoe Wigston¹ and Edward M Vital², ¹University of Leeds, Leeds, United Kingdom, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ³The University of Manchester, Manchester, United Kingdom, ⁴University of Cambridge, Cambridge, United Kingdom
Background/Purpose: Anti-nuclear antibody (ANA) positivity represents a complex ‘At-Risk’ state for development of connective tissue disease (CTD). ANA may become positive years in advance of…
Abstract Number: 1097 • ACR Convergence 2022
Intrinsic STAT1 Deficiency Underlies Proinflammatory Imprint of Naive CD4+ T Cells in Spondyloarthritis
Bilade CHERQAOUI¹, Frederic Cremazy¹, Marc Lauraine¹, Ghazal Shammas², Roula Said-Nahal³, Hendrick Mambu Mambueni⁴, Felicie Costantino², Marine Fourmont², Simon Glatigny², Luiza Maria Araujo² and Maxime Breban², ¹INSERM U1173, UFR Simone Veil, Versailles-Saint Quentin / Paris-Saclay University, Montigny-le-Bretonneux, France, ²1INSERM U1173, UFR Simone Veil, Versailles-Saint Quentin / Paris-Saclay University, Montigny-le-Bretonneux, France, ³Rheumatology Division - Ambroise Pare Hospital, APHP, Boulogne-Billancourt, France, ⁴Genomic Platform of Faculty of Health Simone Veil - UVSQ university, Montigny-le-Bretonneux, France
Background/Purpose: Spondyloarthritis (SpA) development is associated with type 3 immune response activation. In HLA-B27/human β2-microglobulin transgenic rat model (B27-rat), this might at least be related…
Abstract Number: 2171 • ACR Convergence 2022
Serum IFN Score Predicts Long Term Outcome in Limited Cutaneous Systemic Sclerosis
Ranjitha Karanth¹, Giuseppina Abignano², Vishal Kakkar¹, Rebecca Ross¹, Chris Denton³ and Francesco Del Galdo⁴, ¹University of Leeds, Leeds, United Kingdom, ²Istituto Reumatologico Lucano (IReL), Azienda Ospedaliera Regionale San Carlo, Potenza, Italy, ³University College London, London, United Kingdom, ⁴Leeds Institute of Rheumatic and Musculoskeletal Medicine, Faculty of Medicine and Health and NIHR Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Type-I Interferon pathway activation has been associated with severity and progression of diffuse cutaneous SSc (dcSSc). The role of type-I Interferons (IFNs) in limited…
Abstract Number: 0636 • ACR Convergence 2022
Serum Soluble Mediator Signatures of Lupus Nephritis Histological Features and Response to Treatment
Andrea Fava¹, Carla J. Guthridge², Joseph Kheir², Catriona Wagner³, Michelle Petri⁴, Jill Buyon⁵, Betty Diamond⁶, the Accelerating Medicines Partnership (AMP) RA/SLE⁷, Joel Guthridge² and Judith James², ¹Johns Hopkins University, Baltimore, MD, ²Oklahoma Medical Research Foundation, Oklahoma City, OK, ³Oklahoma Medical Research Foundation, Oklahoma, ⁴Johns Hopkins University School of Medicine, Division of Rheumatology, Baltimore, MD, ⁵NYU Grossman School of Medicine, New York, NY, ⁶Feinstein Institutes for Medical Research, Manhasset, NY, ⁷Multiple Insitutions
Background/Purpose: There is a pressing need to identify novel therapeutic approaches and noninvasive biomarkers in lupus nephritis (LN). In this study, we quantified serum soluble…
Abstract Number: 1170 • ACR Convergence 2022
Systemic Sclerosis Dermal Fibroblast-derived Exosomes Trigger a Type 1 Interferon Rresponse in Keratinocytes Through TBK1
Jessica Bryon¹, Christopher Wasson¹, Rebecca Ross¹, Elton Zeqiraj¹ 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: Elevated Type I IFN response is present in blood and affected tissues in systemic sclerosis (SSc) and correlates with disease activity and response to…
Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: 0641 • ACR Convergence 2022
IL-4 Receptor Signaling and IKAROS Transcriptomic Program Maintains Naïve B-cell Quiescence and Antagonizes SLE
John Mountz, Min Gao, Shanrun Lui, Walter Chatham and Hui-Chen Hsu, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Factors that promote B-cell quiescence and homeostasis at the transitional (Tr) and naive stages of B cells to prevent excessive TLR7 and type I…
Abstract Number: 1629 • ACR Convergence 2022
AUR200: An Improved BAFF/APRIL Inhibitor with Increased Potency and Safety for the Treatment of B Cell-Mediated Diseases
Shawn Morales, Jennifer Cross and Robert Huizinga, Aurinia Pharmaceuticals, Inc., Victoria, BC, Canada
Background/Purpose: AUR200 targets both B cell activating factor (BAFF) and a proliferation-inducing ligand (APRIL), which are key mediators in the pathogenesis of B cell-mediated autoimmune…
Abstract Number: 2264 • ACR Convergence 2022
Dysregulated Interferon Gamma Signals Promote Systemic Autoimmunity in STAT1 Gain of Function Syndrome
Andrea Largent¹, Jane Buckner², David Rawlings¹ and Shaun Jackson¹, ¹Seattle Children's Research Institute, Seattle, WA, ²Benaroya Research Institute at Virginia Mason, Seattle, WA
Background/Purpose: Heterozygous gain-of-function mutations in STAT1 promote a complex disorder of immune dysregulation including chronic mucocutaneous candidiasis (CMC) and systemic autoimmunity. However, given widespread expression…
Abstract Number: 0644 • ACR Convergence 2022
Long-lived Plasma Cells in the Lupus Bone Marrow Are Imprinted by Interferon
Jennifer Barnas¹, Diana Alzamareh², Neha Nandedkar-Kulkarni², Jennifer Albrecht², Nida Meednu², Cameron Baker², Andrew McDavid¹ and Jennifer Anolik², ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
Background/Purpose: Systemic lupus erythematous (SLE) is a complex autoimmune disorder with heterogeneous disease presentation and a multi-pronged pathogenesis. Although autoreactive plasma cells (PC) play a…
Abstract Number: 1664 • ACR Convergence 2022
TRIM21 as a Regulator of UVB-Driven IFN Responses in Lupus
Richard Moore, Gantsetseg Tumurkhuu, Daniel Wallace and Caroline Jefferies, Cedars-Sinai Medical Center, Los Angeles, CA
Background/Purpose: Patients with systemic lupus erythematosus (SLE) experience photosensitivity where exposure to ultraviolet light B (UVB) drives lupus flares and systemic symptoms. Although the mechanisms…

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