Abstracts tagged "interferon"

Abstract Number: 2397 • ACR Convergence 2024
Increase of IL10 and IFNa2 Are Associated to Clinical Activity in Systemic Lupus Erythematous Patients
Elena Grau García¹, Marta De la Rubia Navarro², Samuel Leal Rodriguez¹, José Ivorra-Cortés³, Carmen Riesco Barcena⁴, Anderson Huaylla Quispe⁴, Laura Mas Sánchez⁵, Pablo Muñoz Martínez⁶, Daniel Ramos Castro⁴, Alba Torrat noves⁷, Iago Alcantara Alvarez², Belén Villanueva Mañés², Miguel Simeo Vinaixa², Andrés Pérez Hurtado⁴ and Jose A Román-Ivorra⁸, ¹HUP La Fe, Valencia, Spain, ²Rheumatology Department. HUP La Fe, Valencia, Comunidad Valenciana, Spain, ³Hospital Universitario La Fe, PALMA DE MALLORCA, Spain, ⁴Rheumatology Department. HUP La Fe, Valencia, Spain, ⁵Resident at Hospital Universitari i Politecnic La Fe, Valéncia, Spain, ⁶Hospital Universitario y Politècnico La Fe, Sagunto, Spain, ⁷Hospital Universitari i Politècnic La Fe de Valencia, Valencia, Comunidad Valenciana, Spain, ⁸Hospital Universitari i Politècnic la Fe, Valencia, Comunidad Valenciana, Spain
Background/Purpose: Systemic lupus erythematous (SLE) is an autoimmune disease characterized by deregulation of cytokine production. Interferon (IFN) is a proinflamatory cytokine considered as a key…
Abstract Number: 0970 • ACR Convergence 2024
Deubiquitylase (DUB) BRCC36 Isopeptidase Complex (BRISC) Inhibitors Prevent IFNAR1 Deubiquitination to Restore Natural Type I IFN Response in Autoimmunity
Rebecca Ross¹, Poli Adi Narayanna Reddy², Joseph Salvino², Elton Zeqiraj³ and Francesco Del Galdo³, ¹Medicine and Health, University of Leeds, Leeds, United Kingdom, ²The Wistar Institute, Philadelphia, PA, ³University of Leeds, Leeds, United Kingdom
Background/Purpose: Increased Type I IFN activation plays a key role in Scleroderma (SSc), Systemic Lupus Erythematosus and Inflammatory Myositis. Deubiquitylase (DUB) BRCC36 isopeptidase complex (BRISC)…
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: 0092 • ACR Convergence 2024
A New Generation Mesenchymal Stromal Cell (MSC)-based Cell Therapy Using Design of Experiments Halts Pristane Induced Glomerulosclerosis
Hulya Bukulmez¹, Adrienne Dennis², kristine Highland³ and Steven N. Emancipator⁴, ¹MetroHealth Medical Center, Case Western Reserve, Pepper Pike, OH, ²MetroHealth Medical Center, Case Western Reserve, Cleveland, OH, ³Cleveland Clinic Foundation, Cleveland, OH, ⁴Case Western Reserve University, Cleveland, OH
Background/Purpose: MSCs, exposed to a disease-specific cytokine profile, can accurately sense the composition of the inflammatory microenvironment. We have harnessed this adaptive ability of MSCs…
Abstract Number: 1426 • ACR Convergence 2024
Single Cell RNA-Seq Characterization of Circulating Immune Cells in Sjogren’s Syndrome: Comparison to SLE and Rheumatoid Arhtritis
Alejandro Gomez-Gomez¹, yolanda Guillen², Paloma Vela Casasempere³, María Paula álvarez Hernández⁴, Ignacio Brana Abascal⁵, Mónica Fernández Castro⁶, Maria López Lasanta¹, José María Pego-Reigosa⁷, Carlos Marras⁸, María García-Villanueva⁹, Francisco Blanco¹⁰, Patricia Carreira¹¹, Carolina Perez-Garcia¹², Íñigo Rúa-Figueroa¹³, Hugo F Avalos bogado¹, Natalia Boix Martí¹, Eric Kirkegaard-Biosca¹⁴, iago álvarez Sáez¹⁵, Nuria Palau¹⁶, Raül Tortosa¹⁶, Jesús Tornero Molina¹⁷, Antonio Fernandez Nebro¹⁸, Jaime Calvo-Alén¹⁹, Juan Cañete²⁰, Damiana Alvarez-Errico², Ernest Choy²¹, Holger Heyn²², Antonio Julia²³, Sara Marsal barril¹ and Jose Luis Andreu²⁴, and on behalf of SSAD and DocTIS consortia, ¹Hospital Universitari Vall d´Hebron, Rheumatology, Barcelona, Spain, ²Imidomics, Inc, Barcelona, Spain, ³Hospital General Universitario de Alicante , Rheumatology, Alicante, Spain, ⁴Hospital universitario de la Princesa. IIS-Princesa, Rheumatology, Madrid, Madrid, Spain, ⁵Hospital Universitario Central de Asturias, Oviedo, Asturias, Spain, ⁶Hospital Universitario Puerta de Hierro Majadahonda., Madrid, Madrid, Spain, ⁷Galicia Health Service (SERGAS), Vigo, Spain, ⁸Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, Murcia, Spain, ⁹Hospital Ramón y Cajal, MADRID, Madrid, Spain, ¹⁰Complejo Hospitalario Universitario de A Coruña , Rheumatology, A Coruña, Spain, ¹¹Hospital Universitario 12 de Octubre, Madrid, Madrid, Spain, ¹²Hospital del Mar, Barcelona, Spain, ¹³Department of Rheumatology, Hospital Universitario Doctor Negrín, Las Palmas de Gran Canaria, Las Palmas GC, Spain, ¹⁴Hospital Universitari Vall d´Hebron, Ophtalmology, Barcelona, Spain, ¹⁵Hospital Universitari Vall d´Hebron, Maxillofacial Surgery, Barcelona, Spain, ¹⁶Vall d´Hebron Hospital Research Institute, Rheumatology, Barcelona, Spain, ¹⁷Sociedad Española de Reumatologia, Rheumatology, Madrid, Spain, ¹⁸Hospital Regional Universitario Carlos Haya , Rheumatology, Málaga, Spain, ¹⁹Department of Rheumatology, Hospital Araba, Vitoria, Pais Vasco, Spain, ²⁰Hospital Clinic an IDIBAPS, Barcelona, Spain, ²¹Cardiff University School of Medicine, Cardiff, United Kingdom, ²²Centre for Genomic Regulation (CNAG-CRG), National Centre for Genomic Analysis, Barcelona, Spain, ²³Vall d'Hebron Hospital Research Institute, Barcelona, Spain, ²⁴Hospital Universitario Puerta de Hierro Majadahonda., Majadahonda, Spain
Background/Purpose: Primary Sjögren’s Syndrome (pSS) shares with SLE and RA features including their predominance in women, immunological characteristics and genetic risk background. Hence, to dissect…
Abstract Number: 1792 • ACR Convergence 2024
CD14+ Monocytes Demonstrate Subclinical Erythrocyte Uptake in Childhood Onset Systemic Lupus Erythematosus
Lauren Robinson¹, Preetha Balasubramanian², Zurong Wan², Juan Rodriguez Alcazar², Marina Lima Silva Santos², Lynnette Walters³, Jeanine Baisch², Karen Onel⁴, Tracey Wright⁵, Virginia Pascual⁶ and Simone Caielli², ¹Hospital for Special Surgery, New York, NY, ²Weill Cornell Medicine, New York, NY, ³Scottish Rite Hospital for Children, Allen, TX, ⁴HSS, New York, NY, ⁵UT Southwestern, Plano, TX, ⁶Weill Cornell Medical College, New York, NY
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a disease characterized by the presence of auto-antibodies, immune complex deposition, and a robust type I interferon (IFN) signature.…

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