Abstracts tagged "interferon"

Abstract Number: 1770 • ACR Convergence 2024
Assessing S100A4 and Myofibroblast Phenotypes in the Pathogenesis of Cardiac and Cutaneous Neonatal Lupus
Nalani Sachan¹, Christina Firl², Philip Carlucci³, Nicola Fraser², Robert Clancy⁴ and Jill Buyon⁵, ¹NYU Grossman School of Medicine, New York, NY, ²NYU Langone Health, New York, NY, ³New York University School of Medicine, New York, NY, ⁴Columbia University Medical Center, New York, NY, ⁵New York University Grossman School of Medicine, New York, NY
Background/Purpose: Neonatal lupus (NL), driven by fetal exposure to maternal anti-SSA/Ro autoantibodies, typically results in permanent cardiac manifestations in utero and/or a transient rash postnatally.…
Abstract Number: 2423 • ACR Convergence 2024
Associations Between Cytometric Interferon Signature and Clinical Response in a Cohort of Anifrolumab-treated Patients with Lupus
Alice Horisberger¹, Katharina Shaw², Eilish Dillon³, Kathryne Marks⁴, Rochelle Castillo⁵, Ifeoluwakiisi Adejorin⁶, Kimberly Hashemi⁴, Karen Costenbader⁷, Avery LaChance⁸, Ruth Ann Vleugels⁵ and Deepak Rao¹, ¹Brigham and Women's Hospital, Harvard Medical School, Boston, MA, ²Perelman School of Medicine at the University of Pennsylvania, Merion Station, PA, ³University of Massachusetts Boston, Yarmouth, MA, ⁴Brigham and Women's Hospital, Boston, MA, ⁵Brigham and Women's Hospital, Brookline, MA, ⁶Brigham and Woman's Hospital, Harvard Medical School, Boston, MA, ⁷Brigham and Women's Hospital/ Harvard Medical School, Boston, MA, ⁸Brigham and Women's Hospital, Jamaica Plain, MA
Background/Purpose: Anifrolumab, a monoclonal antibody targeting type I interferon (IFN-I) receptor subunit 1, was recently approved for the treatment of systemic lupus erythematosus (SLE) and…
Abstract Number: 0001 • ACR Convergence 2024
Type I Interferon and Anifrolumab Effects on Bone Marrow and Blood Plasma Cells in Systemic Lupus Erythematosus
Diana Alzamareh¹, Nida Pellett², Daria Krenitsky², Wesley Durrett¹, Javier Rangel-Moreno¹, Jennifer Anolik² and Jennifer Barnas¹, ¹University of Rochester, Rochester, NY, ²University of Rochester Medical Center, Rochester, NY
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of pathogenic anti-nuclear antibodies (ANA) by plasma cells (PC). Importantly, long-lived plasma…
Abstract Number: 1150 • ACR Convergence 2024
Increased ISG15 Expression in Peripheral Blood from Patients with Positive Dermatomyositis Related Specific Autoantibodies
Sylvia Raftopoulou¹, Nikolaos Michalakeas¹, Maria Gerochristou², Nikolaos Marketos¹, Charalampos Skarlis¹ and Clio Mavragani³, ¹Molecular and Applied Physiology Unit, Department of Physiology, School of Medicine, National and Kapodistrian University of Athens., Athens, Greece, ²Andreas Syggros Hospital, National and Kapodistrian University of Athens, Athens, Greece, ³Department of Physiology and Pathophysiology, School of Medicine, National and Kapodistrian University of Athens., Athens, Greece
Background/Purpose: Idiopathic inflammatory myopathies (IIM) are characterised by muscle inflammation (myositis) and weakness, impacting various organ systems. Clinical subtypes include dermatomyositis (DM), antisynthetase syndrome (ASyS),…
Abstract Number: 1772 • ACR Convergence 2024
Modeling and Predicting HLH Through Measurement of Immune Synapse Duration, Cytokine Production, and Target Cell Death
Anastasia Frank-Kamenetskii¹, Jemy Varghese², Jeremy Morrissette³, Hannah Klinghoffer⁴, Caroline Diorio⁵, Janis Burkhardt⁶ and Scott Canna², ¹CHOP/UPENN, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia, PA, ³University of Pennsylvania - Perelman School of Medicine, Philadelphia, PA, ⁴The Children's Hospital of Philadelphia, Philadelphia, PA, ⁵Division of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA, ⁶The Children's Hospital of Philadelphia, Philadelphia
Background/Purpose: Hyperinflammation is a life-threatening systemic inflammatory state most commonly associated with Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS), but observed in nearly all inflammatory…
Abstract Number: 2456 • ACR Convergence 2024
High Levels of Circulating IFNα Are Associated with Increased Mortality in Patients with Diffuse Cutaneous Systemic Sclerosis
François Maillet¹, Carine Schmidt¹, Vincent Bondet², Alexandre Bense¹, Darragh Duffy³, Luc Mouthon¹, Mathieu Paul Rodero⁴ and Benjamin Chaigne⁵, ¹AP HP, Paris, France, ²INSERM PAsteur Institute, Paris, France, ³Inserm Pasteur Institue, Paris, France, ⁴Université Paris CIté, Laboratoire de Chimie et de Biochimie Pharmacologiques et Toxicologiques, Faculté des Saint-Pères, Paris, France, ⁵Service de Médecine Interne, Centre de Référence Maladies Systémiques Autoimmunes et Autoinflammatoires Rares d'Ile de France de l’Est et de l’Ouest, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, Ile-de-France, France
Background/Purpose: Systemic sclerosis (SSc) is a connective tissue disease characterized by skin and organ fibrosis, autoimmunity, and vasculopathy. Although its pathophysiology remains unclear, recent data…
Abstract Number: 0020 • ACR Convergence 2024
CD14 Deficiency Protects Against TLR4/LPS-mediated Inhibition of Osteoclastogenesis
Lance Murphy¹, Kevin Burt², Vu Nguyen³, Baofeng Hu², Robert Mauck³ and Carla Scanzello², ¹University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania, Philadelphia
Background/Purpose: Osteoarthritis is associated with bone changes such as subchondral sclerosis, bone marrow lesions, and osteophyte formation (Donel S, 2019). Prior work has demonstrated that…
Abstract Number: 1266 • ACR Convergence 2024
Anti-Mitochondrial Antibodies Associate with Disease Activity and IFNα Expression in Childhood-onset Systemic Lupus Erythematosus
Marina Barguil Macedo¹, Javad Wahadat², Albin Bjoerk³, Sylvia Kamphuis², Marjan Versnel² and Christian Lood¹, ¹University of Washington, Seattle, WA, ²Erasmus University Medical Center, Rotterdam, Netherlands, ³Karolinska Institutet, Stockholm, Sweden
Background/Purpose: Extracellular mitochondria may represent a source of antigenic burden in autoimmune disorders, such as in systemic lupus erythematosus (SLE), in which those organelles, released…
Abstract Number: 1778 • ACR Convergence 2024
A Novel Variant in IRAK2 Results in Immune Dysregulation in Systemic Juvenile Idiopathic Arthritis (sJIA)
Mariana Correia Marques¹, Brooke Boyd², Alana Platukus³, Elizabeth Schmitz², Hiroto Nakano², Faiza Naz⁴, Anthony Cruz¹, Stefania Dell'Orso⁴, Zuoming deng⁴ and Michael Ombrello⁵, ¹National Institutes of Health, Bethesda, MD, ²Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ³1Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, MD, ⁴National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, Bethesda, MD, ⁵National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD
Background/Purpose: Lung disease (LD) is poorly understood complication of Still’s disease with high fatality. HLA-DRB1*15 is a strong risk factor for the development of this…
Abstract Number: 2518 • ACR Convergence 2024
Behçet’s Disease Immune Landscape Exhibits a Universal NF-kB-mediated Hyperactivation Pattern with Cell-specific TNFAIP3 Responses and a Superimposed IFN-regulated Endotype
Yesim Ozguler¹, Olivier Manches², Didar Ucar³, Ziyan Lin², Alireza Khodadadi-Jamayran², Aristotelis Tsirigos², Gulen Hatemi¹ and Johannes Nowatzky⁴, ¹Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²NYU Grossman School of Medicine, New York, NY, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Ophthalmology, Istanbul, Turkey, ⁴New York University Grossman School of Medicine, New York, NY
Background/Purpose: The immune landscape of Behçet’s disease (BD) remains ill-defined, lacking a unifying picture, precise disease phenotype-endotype correlations, and an understanding of immunity at target…
Abstract Number: 0762 • ACR Convergence 2023
Liver Disease Is a Common Feature of HA20 That Causes Significant Morbidity Associated with Interferon Induction
Magdalena Harasimowicz¹, Deborah Stone², Manuel Carpio Tumba¹, Tina Romeo², Urekha Karri¹, Patrycja Hoffmann³, Helen Leavis⁴, Alexander Miethke⁵, Theo Heller², Anjali Rai², Ivona Aksentijevich², amanda ombrello⁶, Daniel Kastner⁷ and Daniella Schwartz¹, ¹University of Pittsburgh, Pittsburgh, PA, ²NIH, Bethesda, MD, ³NIH, Vienna, VA, ⁴Infection Immunity Utrecht, Utrecht, Netherlands, ⁵Cincinnati Children's Hospital, Cincinnati, OH, ⁶National Institutes of Health, Rockville, MD, ⁷National Human Genome Research Institute, Bethesda, MD
Background/Purpose: Heterozygous loss-of-function TNFAIP3 mutations cause A20 haploinsufficiency (HA20), an early-onset immune dysregulatory disease1. While HA20 was initially described as an inherited form of Behcet’s…
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…

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