Abstracts tagged "interferon"

Abstract Number: 0937 • ACR Convergence 2023
IL-4+ and IFN-α+ Profibrotic T Cells Aggravate Systemic Sclerosis via STIM1/STING Signaling in SKG Mice
Ji Won Yang¹, Kun Hee Lee², Jin Seok Woo², Ha Yeon Jeong², JeongWon Choi², Eui-Jong Kwon¹, Mi-La Cho² and Sung-Hwan Park¹, ¹Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea, ²Rheumatism Research Center, Catholic Research Institute of Medical Science, College of Medicine, The Catholic University of Korea, Seoul, South Korea
Background/Purpose: Systemic sclerosis (SS), also known as scleroderma, is a chronic autoimmune disease caused by inflammation, tissue fibrosis, and vasculopathy. Although the pathological mechanisms of…
Abstract Number: 2044 • ACR Convergence 2023
Monogenic Interferon Mediated Diseases: Novel Phenotype and Genotype Characteristics from Saudi Population
Alhanouf Alsaleem, Sulaiman Al-Mayouf and Shahad Alansari, King Faisal Specialist Hospital & Research Centre, Riyadh, Saudi Arabia
Background/Purpose: IFN-mediated diseases are mendelian innate immunodysregulatory disorders that present early in life with fevers, sterile organ inflammation, and a high type-I IFN-response gene signature…
Abstract Number: 0574 • ACR Convergence 2023
Type I IFN-associated Regulation of Immune Checkpoint Genes in Patients with SLE
Kanwal Zahid Siddiqi¹, Amanda Hempel Zinglersen², Katrine Kjær Iversen¹ and Søren Jacobsen², ¹Copenhagen Research Center for Autoimmune Connective Tissue Diseases (COPEACT), Department of Rheumatology 4242, Rigshospitalet, University Hospital of Copenhagen, Copenhagen, Denmark, ²Copenhagen Research Center for Autoimmune Connective Tissue Diseases, Center for Rheumatology and Spine Diseases, Copenhagen University Hospital, Rigshospitalet, Copenhagen, Denmark
Background/Purpose: Aberrant activation of type I IFN, and subsequent adverse clinical implications, have been widely discussed in relation to SLE. With the lack of consistent…
Abstract Number: 1176 • ACR Convergence 2023
Brepocitinib Prevents Type-I Interferon Induced Damage in Cultured Myocytes and Endothelial Cells Indicating a Potential Role in the Treatment of Dermatomyositis
Jiri Vencovsky¹, Jolie Feldman², Lisa McConnachie² and Brendan M. Johnson², ¹Institute of Rheumatology, Prague, Czech Republic, Department of Rheumatology, 1st Faculty of Medicine, Charles University, Prague, Czech Republic, ²Priovant Therapeutics, Durham, NC
Background/Purpose: Dermatomyositis (DM), an idiopathic inflammatory myopathy, is a chronic and often debilitating condition characterized by a hallmark skin rash (e.g., Gottron's sign and heliotrope…
Abstract Number: 2297 • ACR Convergence 2023
Interferon-α as a Biomarker to Predict Flares in Lupus Nephritis
Laura Patricia Whittall Garcia¹, Dafna Gladman², Murray Urowitz³, Zahi Touma⁴ and Joan Wither¹, ¹University Health Network, Toronto, ON, Canada, ²Schroeder Arthritis Institute, Krembil Research Institute, Toronto Western Hospital, Department of Medicine, University of Toronto, Toronto, ON, Canada, ³Schroeder Arthritis Institute, Krembil Research Institute; University of Toronto Lupus Clinic; Division of Rheumatology, Toronto, ON, Canada, ⁴University of Toronto, Toronto, ON, Canada
Background/Purpose: Type I Interferons (IFN-I) play a role in SLE and Lupus Nephritis (LN) pathogenesis. We have recently shown that IFN-I gene expression predicts the…
Abstract Number: 0598 • ACR Convergence 2023
Litifilimab Modulates Type I IFN Biomarkers in Patients with SLE or CLE in the Phase 2 LILAC Study
Richard Furie¹, Victoria Werth², Eric Milliman³, Kyle Ferber³, Fergal Casey³, Roland Brown³, Denitza Raitcheva³, Jad Zoghbi³, Danielle Graham³, George Kong³, Youmna Lahoud³, Nathalie Franchimont⁴ and Catherine Barbey⁵, ¹Northwell Health, Manhasset, NY, ²University of Pennsylvania and Corporal Michael J. Crescenz VAMC, Philadelphia, PA, ³Biogen, Cambridge, MA, ⁴Former employee of Biogen, Cambridge, MA, ⁵Biogen, Baar, Switzerland
Background/Purpose: Litifilimab is a humanized IgG1 mAb targeting BDCA2, a receptor expressed on plasmacytoid dendritic cells (pDCs), that negatively regulates the production of Type I…
Abstract Number: 1240 • ACR Convergence 2023
Obvious Only in Retrospect: A Cohort of STING Associated Vasculopathy in Infancy (SAVI) Without Typical Rash
Ilaria Maccora¹, Patricia Vega-Fernandez², Kimberly Risma³ and Grant Schulert⁴, ¹PhD student, in the Area of Drugs and Innovative Treatments, NeuroFARBA Department, University of Florence. Meyer Children's Hospital, Firenze, Italy, ²Division of Rheumatology, Cincinnati Children's Hospital, Medical Centre, Cincinnati, OH, ³Division of Allergy and Immunology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is characterized by systemic inflammation, skin vasculopathy and interstitial lung disease. However, since the initial description knowledge…
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…

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