Abstracts tagged "interferon"

Abstract Number: 1587 • ACR Convergence 2023
Mitochondrial Z-DNA and ZBP1 Drive Autoimmune Photosensitivity
Benjamin Klein, Mack Reynolds, Bin Xu, Mehrnaz Gharaee-Kermani, Amanda Victory, Shannon Loftus, Mary O'Riordan and J. Michelle Kahlenberg, University of Michigan, Ann Arbor, MI
Background/Purpose: Autoimmune photosensitivity is observed in type I Interferon (IFN) mediated diseases such as systemic and cutaneous lupus erythematosus (SLE/CLE) and dermatomyositis. Type I IFN…
Abstract Number: 0788 • ACR Convergence 2023
Whole Exome Sequencing and Evolutionary Action Missense Mutation Analysis Identifies MICB as a New SSc Susceptibility Locus and the Interferon Pathway as Contributors to SSc Pathogenesis
Shamika Ketkar¹, Hongzheng Dai¹, Brian Dawson¹, Lindsay Burrage¹, David Murdock¹, Jennifer Asmussen¹, Kevin Wilhelm¹, Olivier Lichtarge¹, Martin Kerick², Javier Martin³, Shervin Assassi⁴, Brendan Lee¹ and Maureen Mayes⁵, ¹Baylor College of Medicine, Houston, TX, ²Spanish National Research Council, Granada, Spain, ³Instituto de Parasitologia y Biomedicina Lopez-Neyra - CSIC, Granada, Spain, ⁴University of Texas McGovern Medical School at Houston, Houston, TX, ⁵Division of Rheumatology, University of Texas McGovern Medical School, Houston, TX
Background/Purpose: Systemic Sclerosis (SSc) is characterized by fibrosis of the skin and internal organs associated with vasculopathy and autoantibodies and exhibiting wide clinical heterogeneity. Contributors…
Abstract Number: 1590 • ACR Convergence 2023
Autoimmune Pathway Blockade by a Potent Orally Bioavailable STING Antagonist
Min Yang¹, Hailong Li¹, yangyang liu¹, lili yao¹, Jing Lin¹, Zhi xie² and Wenge zhong¹, ¹Regor Therapeutics, Shanghai, China, ²Regor Pharmaceuticals, Inc., San Francisco, CA
Background/Purpose: The cyclic GMP-AMP synthase (cGAS)-stimulator of interferon genes (STING) pathway has emerged as a key innate immune mediator of autoimmune and inflammatory diseases. However,…
Abstract Number: 0792 • ACR Convergence 2023
Single Cell RNA-seq of Myeloid Cells from Systemic Sclerosis Patients Identifies Circulating Monocyte Population with Interferon Signature Associated with Interstitial Lung Disease
Richard Ainsworth¹, Kim Taylor², Ye Cao³, Takanori Sasaki⁴, Deepak Rao⁵, Nunzio Bottini⁶ and Francesco Boin¹, ¹Cedars-Sinai Medical Center, Los Angeles, CA, ²University of California San Francisco, San Francisco, CA, ³Brigham and Women's Hospital, Boston, MA, ⁴Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁵Brigham and Women's Hospital, Boston, MA, ⁶University of California, San Francisco, CA
Background/Purpose: Growing evidence supports a critical role for innate immunity in systemic sclerosis (SSc) pathogenesis. Altered myeloid cell numbers and functions have been implicated in…
Abstract Number: 1658 • ACR Convergence 2023
Title: Inflammatory Arthritis Across the Age-Spectrum: Single-Cell Profiling of the Inflamed Synovium in Children with Juvenile Idiopathic Arthritis
Chrissy Bolton¹, Christopher Mahony², Charlotte Smith², Vicky Alexiou³, Huong Nguyen³, Patricia Reis-Nisa², Søren Lomholt⁴, Annie Hackland², Sugrah Sultan⁵, Klaudia Kupiec³, Sunit Davda⁶, Charlene Foley⁶, Calliope Dendrou¹, Elizabeth C Rosser⁷, Accelerating Medicines Partnership Program RA SLE Network⁸, Anna Helena Jonsson⁹, Fan Zhang¹⁰, Michael Brenner¹¹, Soumya Raychaudhuri⁹, Christopher Buckley¹, Manigandan Thyagarajan⁵, Zishan Shiekh⁵, Sandrine Compeyrot-Lacassagne⁶, Samantha Chippington⁶, Mark Coles¹, Eslam Al-Abadi⁵, Andrew Filer², Tissue Research in Childhood Onset Inflammatory Arthritis (TRICIA) Consortium¹², Lucy R Wedderburn³ and Adam Croft², ¹University of Oxford, Oxford, United Kingdom, ²University of Birmingham, Birmingham, United Kingdom, ³UCL Great Ormond Street Institute of Child Health, London, United Kingdom, ⁴Aarhus University, Aarhus, Denmark, ⁵Birmingham Women’s and Children’s NHS Foundation Trust, Birmingham, United Kingdom, ⁶Great Ormond Street Hospital, London, United Kingdom, ⁷University College London, London, United Kingdom, ⁸Cedars-Sinai Medical Center, Los Angeles, CA, ⁹Brigham and Women's Hospital, Boston, MA, ¹⁰University of Colorado, Aurora, CO, ¹¹Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ¹²MRC, Birmingham, United Kingdom
Background/Purpose: Understanding the unique and shared pathogenic processes between childhood-onset and adult-onset inflammatory arthritides is needed to guide more effective drug development and their application…
Abstract Number: 0010 • ACR Convergence 2023
A Potential Mechanism for Major Adverse Cardiac Events Associated with JAK Inhibitors: JAK Inhibitor Withdrawal Causes Urokinase Release by Primed STAT Signaling
Sara McCoy¹, Ilya Gurevic² and jacques Galipeau², ¹University of Wisconsin School of Medicine and Public Health, Middleton, WI, ²University of Wisconsin, Madison, WI
Background/Purpose: Sjögren’s Disease (SjD) has high glandular IFNg levels, associated with disease activity and lymphoma risk. We showed that IFNg-stimulated minor salivary gland (SG)-mesenchymal stromal…
Abstract Number: 0886 • ACR Convergence 2023
Neonatal Roseolovirus Infection Predisposes to Development of Lupus-like Disease After TLR7 Stimulation
Tarin Bigley¹, Eden Xue¹, Lifei Zhu¹, Li-ping Yang¹ and wayne Yokoyama², ¹Washington University in St. Louis, St. Louis, MO, ²Washington University School of Medicine, St. Louis, MO
Background/Purpose: Viral infections have been implicated as major factors in autoimmune disease but demonstrating causality is often challenging. We found that neonatal infection with a…
Abstract Number: 1660 • ACR Convergence 2023
Still’s Disease Patients with High Interferon-stimulated Gene Expression Have Enrichment of Rare, de Novo and Recessive Protein Altering Variants in Innate Immune Pathways
Mariana Correia Marques¹, Zuoming Deng², Navid Chowdhury², Elizabeth Schmitz³, Alana Platukus⁴, Stephen Brooks⁵, Carol Lake², Ly-Lan Bergeron², Michelle Millwood² and Michael Ombrello⁶, ¹National Institute of Arthritis & Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), St. Louis, MO, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Philadelphia, PA, ⁵Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skins Diseases, National Institutes of Health, Bethesda, MD, ⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Still’s disease (systemic juvenile idiopathic arthritis in children, adult-onset Still’s disease in adults) is an enigmatic inflammatory condition that affects people of all ages.…
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: 0158 • ACR Convergence 2022
Effect of Type I IFN on Mitochondria and Muscle Weakness in Myositis
Melissa Morales, Joanna Parkes, Sabrina Narvesen and Rita Spathis, Department of Pharmaceutical Sciences, Binghamton University, Binghamton, NY
Background/Purpose: Muscle weakness is a hallmark of autoimmune myositis. The mechanisms that contribute to muscle weakness are currently unknown. The observed ineffectiveness after immunomodulatory treatment…
Abstract Number: 0663 • ACR Convergence 2022
Anti-Erythrocyte Antibodies in Childhood Onset Systemic Lupus Erythematosus
Lauren Robinson¹, Zurong Wan², Preetha Balasubramanian³, Juan Rodriguez Alcazar³, Lynnette Walters⁴, Jeanine Baisch², karen onel⁵, Tracey Wright⁶, Virginia Pascual³ and Simone Caielli³, ¹Hospital for Special Surgery/New York Presbyterian-Weill Cornell, New York, NY, ²Weill Cornell Medicine, New York, NY, ³Weill Cornell Medical College, New York, NY, ⁴Scottish Rite Hospital for Children, Allen, TX, ⁵Hospital for Special Surgery, New York, NY, ⁶UT Southwestern, Plano, TX
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a disease characterized by the presence of auto-antibodies, immune complex deposition, and a robust type 1 interferon signature. It…
Abstract Number: 1745 • ACR Convergence 2022
Effects of Excess IL-18 in Mixed Inflammatory Environments
Vinh Dang¹, Jemy Varghese¹, Emily Landy², Lauren Van Der Kraak², Leonardo Huang¹, Anastasia Frank-Kamenetskii¹ and Scott Canna¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Interleukin 18 (IL-18) is an inflammasome-activated, IL-1 family cytokine that canonically induces interferongamma (IFNg). IL-18 activity is potently inhibited by a soluble, IFNg-inducible antagonist,…
Abstract Number: 0324 • ACR Convergence 2022
Comparison of NCounter® and BioFire® Technologies for the Measurement of Type I Interferon Signature
Lena Kolb¹, Marine Mommert², Karen Brengel-Pesce², Roberta Alexander¹, Vasileios Kyttaris³, Anja Kammesheidt⁴ and Geoffrey Stephens¹, ¹Exagen, Inc., Vista, CA, ²bioMérieux, Lyon, France, ³Beth Israel Deaconess Medical Center, Boston, MA, ⁴self, Laguna Beach, CA
Background/Purpose: It is known that the expression of type I Interferon (IFN) genes is upregulated in systemic lupus erythematosus (SLE). In this study, we determined…

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