ACR Meeting Abstracts

ACR Meeting Abstracts

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Abstracts tagged "interferon"

  • 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…
  • Abstract Number: 0668 • ACR Convergence 2022

    Differential Expression of Interferon Related Genes in SLE Patients of Asian and European Ancestries Abstract

    Ian Rector1, Prathyusha Bachali2, Jinoos Yazdany3, Maria Dall'Era4, Amrie Grammer5 and Peter lipsky1, 1AMPEL Biosolutions, Charlottesville, VA, 2AMPEL BioSolutions, Redmond, WA, 3UCSF, San Francisco, CA, 4University of California, Division of Rheumatology, San Francisco, CA, 5AMPEL LLC, Charlottesville, VA

    Background/Purpose: Interferon has been shown to play a role in the pathogenesis of SLE, but insufficient studies have been conducted into the differences in interferon…
  • Abstract Number: 1862 • ACR Convergence 2022

    Lenabasum Reduces IFNγ and pIRF3 in Dermatomyositis Skin: Biomarker Results from a Double-Blind Phase 3 International Randomized Controlled Trial

    Thomas Vazquez1, Meena Sharma1, Rui Feng2, DeAnna Diaz3, Nilesh Kodali1, Josh Dan3, Madison Grinnell3, Emily Keyes3, Grant Sprow3, Barbara White4 and Victoria Werth3, 1Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 2University of Pennsylvania, Philadelphia, 3Philadelphia VAMC, Philadelphia, PA, USA and Department of Dermatology, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, 4Corbus Pharmaceuticals, Pleasanton, CA

    Background/Purpose: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily affects the skin and lung; few effective treatment options are available. Lenabasum is a…
  • Abstract Number: 0359 • ACR Convergence 2022

    Attainment of Remission with Anifrolumab: A Post Hoc Analysis of Pooled TULIP-1 and TULIP-2 Datasets

    Ronald Van Vollenhoven1, Eric F. Morand2, Richard A. Furie3, Ian N. Bruce4, Gabriel Abreu5, Raj Tummala6, Hussein Al-Mossawi7 and Catharina Lindholm8, 1Department of Rheumatology, Amsterdam Rheumatology and Immunology Center, University of Amsterdam, Amsterdam, Netherlands, 2Monash University, Melbourne, Australia, 3Northwell Health, Great Neck, NY, 4The University of Manchester, Manchester, United Kingdom, 5AstraZeneca, Mölndal, Sweden, 6AstraZeneca, Gaithersburg, MD, 7AstraZeneca, Cambridge, United Kingdom, 8AstraZeneca, Gothenburg, Sweden

    Background/Purpose: In patients with SLE, achieving remission is a treat-to-target goal. Remission is associated with lower rates of hospitalization and damage accrual and better quality…
  • Abstract Number: 0669 • ACR Convergence 2022

    Understanding How Type I Interferon Modulates Langerhans Cell ADAM17 to Promote Photosensitivity in Lupus

    Thomas Li1, Ethan Seltzer1, Victoria Zyulina1, Keila Veiga2, Noa Schwartz3, Yurii Chinenov1, David Oliver1, Jose Lora4, Ali Jabbari5, Yong Liu1, Hafsa Munir1, William Shipman1, Marvin Sandoval1, Isabel Sollohub1, william ambler1, Bikash Mishra4, sarah taber1, karen onel1, Mehdi Rashighi6, james krueger7, Niroshana Anandasabapathy4, Carl Blobel1 and theresa Lu1, 1Hospital for Special Surgery, New York, NY, 2Maria Fareri Children's Hospital, New York, NY, 3Albert Einstein College of Medicine, New York, NY, 4Weill Cornell Medicine, New York, NY, 5University of Iowa, Iowa City, IA, 6University of Massachusetts Medical School, Worcester, MA, 7Rockefeller University, New York, NY

    Background/Purpose: Photosensitivity is a hallmark symptom of lupus erythematosus (LE), in which patients develop inflammatory skin lesions in response to ultraviolet radiation (UVR). In examining…
  • Abstract Number: 1879 • ACR Convergence 2022

    Analysis of the Association Between the Atrophic Factors Tripartite Motif Containing (TRIM) 63 and Atrogin-1 and the Clinical and Inflammatory Features of Patients with Idiopathic Inflammatory Myopathies

    jiram torres-Ruiz1, Abdiel Absalón-Aguilar2, Juan Alberto Reyes-Islas2, Alfredo Pérez-Fragoso2, Nancy R Mejía-Domínguez3, guillermo Juárez-Vega4, Alejandro Alfaro-Goldaracena5, Beatriz Alcalá-Carmona2, Guillermo Juárez-Vega3, Fabiola Cassiano-Quezada2 and Diana Gómez-Martín1, 1INCMNSZ, Ciudad de México, Mexico, 2Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, Mexico, 3Red de Apoyo a la Investigación, Universidad Nacional Autónoma de México, Ciudad de México, Mexico, 4Red de Apoyo a la Investigación. UNAM, Ciudad de México, Mexico, 5Department of Surgery, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Ciudad de México, Mexico

    Background/Purpose: Muscle atrophy is mediated by the ubiquitination of myofilaments by two ubiquitin ligases called Tripartite Motif Containing (TRIM) 63 and Atrogin-1, which are induced…
  • Abstract Number: 0360 • ACR Convergence 2022

    Longitudinal Variation of Proteomic Biomarkers That Correlate with Efficacy Endpoints: Results from a Phase 3 Trial of Anifrolumab in Moderate to Severe Systemic Lupus Erythematosus

    Mark Lazarus1, Paul Newcombe1, Richard A. Furie2, Philip Brohawn3, Wendy White3, Dominic Sinibaldi3, Nicola Ferrari1, Raj Tummala3, Hussein Al-Mossawi1, Edward Vital4, Eric F. Morand5, Daniel Muthas6 and Madhu Ramaswamy3, 1AstraZeneca, Cambridge, United Kingdom, 2Northwell Health, Great Neck, NY, 3AstraZeneca, Gaithersburg, MD, 4University of Leeds, Leeds, England, United Kingdom, 5Monash University, Melbourne, Australia, 6AstraZeneca, Gothenburg, Sweden

    Background/Purpose: Phase 2/3 clinical trials in patients with moderate to severe SLE have demonstrated that anifrolumab, a monoclonal antibody blocking IFNAR1, produced better clinical outcomes…
  • Abstract Number: 0740 • ACR Convergence 2022

    Real-World Demographics, Clinical Characteristics, and Treatment Patterns of Patients Treated with Emapalumab for Secondary Hemophagocytic Lymphohistiocytosis in the United States: The REAL-HLH Study

    Carl Allen1, Shanmuganathan Chandrakasan2, Michael Jordan3, Jennifer Leiding4, Abiola Oladapo5, Priti Pednekar6, Kelly Walkovich7 and John Yee8, 1Baylor College of Medicine, Houston, TX, 2Emory University School of Medicine, Atlanta, GA, 3University of Cincinnati, Cincinnati, OH, 4Johns Hopkins University, Baltimore, MD, 5Swedish Orphan Biovitrum AB, Boston, MA, 6PRECISIONheor, Los Angeles, CA, 7University of Michigan Health, Ann Arbor, MI, 8Sobi - North America, Waltham, MA

    Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome caused by overproduction of proinflammatory cytokines, e.g., interferon gamma (IFNγ). Secondary HLH (sHLH), a subtype…
  • Abstract Number: 1931 • ACR Convergence 2022

    NOD2 Mutations Are Associated with Upregulated Type 1 Interferon Gene Expression and Development of Granulomatous Hepatitis in Children with Autoimmune Hepatitis

    Esraa Eloseily1, Alexander Valencia2, Astha Malik2, Rebekah Karns2, Cyd Castro Rojas2, Mosab Alquraish2, Rebecca Marsh3, Alexei Grom4 and Alexander Miethke2, 1Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Division of Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 3Division of Bone Marrow Transplantation & Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Mutations in the gene NOD2 encoding the nucleotide-binding oligomerization domain-containing protein 2 (NOD2) which controls innate responses to LPS have been linked to Blau…
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