Abstracts tagged "interferon"

Abstract Number: 0951 • ACR Convergence 2024
A Feedback Loop Involving mtDNA, Platelets and Neutrophil Extracellular Traps Amplifies Inflammation in Systemic Sclerosis
Stavros Giaglis¹, Diego Kyburz² and Ulrich Walker³, ¹Experimental Rheumatology Laboratory, University of Basel, Basel, Switzerland, ²University Hospital Basel, Basel, Switzerland, ³Basel University Hospital, Basel, Switzerland
Background/Purpose: Mitochondria are essential eucaryotic cell organelles with several bacterial features, such as a double-stranded circular genome with hypomethylated CpG areas (1). A fundamental role of…
Abstract Number: 1766 • ACR Convergence 2024
STAT2-Associated Type I Interferonopathy: A Masquerade of Infectious Susceptibility
Conor Gruber¹, Angelica Lee², Sofija Buta², Marta Martin Fernandez², Veronique Houdouin³, Jean-Laurent Casanova⁴, Alice Hadchouel⁵, Jacinta Bustamante⁵ and Dusan Bogunovic², ¹Mount Sinai Hospital, New York, NY, ²Columbia University, New York, NY, ³Robert Debré Hospital, Paris, France, ⁴Rockefeller University, New York, NY, ⁵Hôpital Necker-Enfants Malades, Paris, France
Background/Purpose: Type I IFN (IFN-I) signaling is a potent inflammatory pathway fundamental to antiviral immunity. In humans, loss of IFN-I activity underlies severe viral disease,…
Abstract Number: 2385 • ACR Convergence 2024
Increased Type I Interferon Inducible Genes Expression in the Peripheral Blood of Patients Presenting Cutaneous Lupus Erythematosus Manifestations
Anastasia- Bilio Chronopoulou¹, Maria Gerochristou², Sylvia Raftopoulou³, Alexander Stratigos⁴ and Clio Mavragani⁵, ¹National and Kapodistrian University of Athens, Athens, Greece, ²Andreas Syggros Hospital, National and Kapodistrian University of Athens, Athens, Greece, ³Molecular and Applied Physiology Unit, Department of Physiology, School of Medicine, National and Kapodistrian University of Athens., Athens, Greece, ⁴1st Department of Dermatology-Venereology, Faculty of Medicine, Andreas Sygros Hospital, National and Kapodistrian University of Athens, Athens, Greece, ⁵Department of Physiology and Pathophysiology, School of Medicine, National and Kapodistrian University of Athens., Athens, Greece
Background/Purpose: Cutaneous Lupus Erythematosus (CLE) is a chronic autoimmune skin disease characterized by a spectrum of cutaneous manifestations resulting from immune dysregulation and inflammation. While…
Abstract Number: 0954 • ACR Convergence 2024
Increased Proliferating Natural Killer Cells Exhibit an Aberrant Pro-Inflammatory Gene Signature in Systemic Sclerosis Patients
Pietro Bearzi¹, Elena Pachera², Sophie Wagner³, Astrid Hofman⁴, Lumeng Li⁴, Laura Much⁴, Mike Becker⁵, Kristina Bürki⁴, Luca Navarini⁶, Anna-Maria Hoffmann-Vold⁷, Roberto Giacomelli⁸ and Oliver Distler⁹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Schlieren, Switzerland, ²University Hospital Zurich, Zurich, Switzerland, ³University of Zurich, Schlieren, Switzerland, ⁴Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, ⁵Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ⁶Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Rome, Italy, ⁷Oslo University Hospital, Oslo, Norway, ⁸Rheumatology, Immunology and Clinical Medicine Unit, Department of Medicine, University of Rome "Campus Bio-Medico", Roma, Italy, ⁹Department of Rheumatology, University Hospital Zurich, University of Zurich, Zurich, Switzerland, Zurich, Switzerland
Background/Purpose: Recent transcriptomic data suggest a prominent involvement of innate immune cells in the pathogenesis of SSc. In this regard, contrasting data on NK cells…
Abstract Number: 1768 • ACR Convergence 2024
A Potential Role of Longstanding IL-18 Stimulation in the Susceptibility for Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Greta Rogani¹, Remco Erkens¹, Marein Putmans¹, Rianne Scholman¹, Jorg van Loosdregt² and Sebastiaan Vastert¹, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²University Medical Center Utrecht, La Jolla, CA
Background/Purpose: Macrophage Activation Syndrome (MAS) is a pathologic condition of immune hyperactivation, which occurs in 10-30% of cases of Still’s Disease (SD), the spectrum of…
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…

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