Abstracts tagged "interferon"

Abstract Number: 1706 • ACR Convergence 2024
Multi-omic Study in Patients with SITRAME Syndrome
Yixiang Yves-Jean Zhu¹, Angèle Soria², Thomas Moreau³, Guilaine Boursier⁴, Vincent Bondet⁵, Françoise Donnadieu⁶, Clara Cretet⁷, Aness Haddouche⁷, Carine Schmidt³, Diego Bletry³, Emmanuelle Amsler², Annick Barbaud², Farah Rahal⁵, Yannick Chantran⁸, Margaux Cescato³, François Maillet⁹, Anne-Sophie Korganow¹⁰, Benjamin Chaigne¹¹, Yannick Dieudonné¹⁰, Guillaume Lefevre¹², Makoto Miyara⁷, Vivien Beziat¹³, Caroline Deswarte¹³, Michael White⁶, Sophie Georgin-lavialle¹, Darragh duffy⁵ and Mathieu Paul Rodero³, ¹Sorbonne Université, Department of internal medicine, Tenon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ²Sorbonne Université, Department of dermatology and allergology, Tenon hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ³Université Paris CIté, Laboratoire de Chimie et de Biochimie Pharmacologiques et Toxicologiques, Faculté des Saint-Pères, Paris, France, ⁴University of Montpellier, Montpellier, ⁵Translational Immunology Unit, Institut Pasteur, Université Paris-Cité, Paris, France, Paris, France, ⁶Pasteur Institut, Laboratoire d'épidémiologie et analyse des maladies infectieuses, Paris, France, ⁷Sobonne University, Centre d'Immunologie et des Maladies Infectieuses, Paris, France, ⁸Departmen of Biological Immunology, Saint-Antoine Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France, ⁹AP HP, Paris, France, ¹⁰University of Strasbourg, Department of Clinical Immunology and Internal Medicine, Nouvel Hôpital Civil, Strasbourg, France, ¹¹Service 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, ¹²CHU Lille, Institut d’Immunologie, Lille, France., Lille, France, ¹³IMAGINE Institut, Human Genetics of Infectious Diseases Laboratory, Paris, France
Background/Purpose: The SITRAME syndrome (Systemic Inflammatory Trunk Recurrent Acute Macular Eruption) is a newly described inflammatory entity affecting adult patients with no family history (Soria…
Abstract Number: 2199 • ACR Convergence 2024
Mycobacterial Infection and Renal and Bladder Malignancy in 2 IFNopathy Patients on High Doses of JAK Inhibitors
Sara Alehashemi¹, Kader Cetin Gedik², Cassandra Calabrese³, Sherry Nguyen⁴, Alexi Baumgardner⁴, Katsiaryna Uss⁴, Kip Friend⁴, Ariane Soldatos⁵, Adriana Almeida de Jesus⁶ and Raphaela Goldbach-Mansky⁷, ¹NIH/NIAID/TADS, Potomac, MD, ²Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Pittsburgh, PA, ³Cleveland Clinic Foundation, Cleveland Heights, OH, ⁴Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Bethesda, MD, ⁵NINDS/NIH, Bethesda, MD, ⁶NIAID, NIH, Silver Spring, MD, ⁷Translational Autoinflammatory Diseases section (TADS), LCIM, NIAID, NIH, Potomac, MD
Background/Purpose: Autoinflammatory Type I Interferonopathies (IFNopathies) include STINGopathies (e.g., SAVI and COPA syndrome), proteasomopathies (e.g., CANDLE/Proteasome associated autoinflammatory syndrome (PRAAS), and oligonucleotidopathies (e.g., AGS: Aicardi-Goutières…
Abstract Number: 0901 • ACR Convergence 2024
Intergenic Alu Elements Are Uniquely Expressed in Dermatomyositis and Correlate with Interferon Stimulated Genes
Rayan Najjar¹, Andrew Mammen² and Tomas Mustelin¹, ¹University of Washington, Seattle, WA, ²NIH, Bethesda, MD
Background/Purpose: As genes constitute < 2% of our genomes, there is a need to explore potential roles of other genomic elements in autoimmune disease. We…
Abstract Number: 1732 • ACR Convergence 2024
In Vitro Screening of siRNAs Designed to Knockdown Interferon Beta as a Novel Therapeutic Approach for Treatment of Adult and Juvenile Dermatomyositis
Joanna Parkes, Andrés Correa-Sánchez, Mark Cunningham and Peter Oliver, MRC Nucleic Acid Therapy Accelerator, Didcot, United Kingdom
Background/Purpose: The main treatments for adult and juvenile dermatomyositis (DM/JDM) are immunosuppressant drugs and corticosteroids, which have significant side effects and are not effective in…
Abstract Number: 2288 • ACR Convergence 2024
Interferon Gamma Signaling Is Associated with Development of Extraglandular Manifestations in Sjögren’s Disease
Hiroyuki Nakamura¹, Koki Nakamura¹, Hidenori Amaike¹, Ken Nagahata¹, Masatoshi Kanda² and Hiroki Takahashi¹, ¹Sapporo Medical University, Sapporo, Japan, ²Department of Rheumatology and Clinical Immunology, Sapporo Medical University, Sapporo, Japan, Sapporo, Japan
Background/Purpose: Sjögren’s disease (SjD) is an autoimmune disease that primarily affects salivary and lacrimal glands. Some patients with SjD develop various extraglandular manifestations (EGM), such…
Abstract Number: 0919 • ACR Convergence 2024
The Effects of CB2R Activation on Inflammatory Pathways in Dermatomyositis
Rohan Dhiman¹, Ahmed Eldaboush², Navin Vijayarangan³, Darae Kang³, Caroline Stone², Nilesh Kodali³, DeAnna Diaz³ and Victoria Werth⁴, ¹University of Pennsylvania, Newport Coast, CA, ²University of Pennsylvania, Philadelphia, PA, ³PennMedicine, Philadelphia, ⁴Hospital of the University of Pennsylvania, Philadelphia, PA
Background/Purpose: Previous in vitro investigations done by our group into the utility of CB2R activation to treat dermatomyositis (DM) used stimulants that activated pathways not…
Abstract Number: 1762 • ACR Convergence 2024
Multi-omic Analysis Defines Heterogeneous and Cell Specific Type I Interferon Signalling in Juvenile-onset SLE Patients Associated with Biomarkers of Cardiovascular Risk
Junjie Peng, Thomas McDonnell, Elizabeth Jury, Coziana Ciurtin and George Robinson, University College London, London, United Kingdom
Background/Purpose: Juvenile-onset systemic lupus erythematosus (jSLE) is an autoimmune disease characterised by chronic inflammation, multi-organ damage, and/or death. Despite their young age, jSLE patients have…
Abstract Number: 2376 • ACR Convergence 2024
Systemic Lupus Erythematosus Patients Exhibit Dynamic Changes in Disease-Associated Transcripts Following Total Knee or Total Hip Arthroplasty
David Fernandez¹, Stephen Batter¹, Deanna Jannat-Khah¹, Mikhail Olferiev¹, Insa Mannstadt², Mark Figgie¹, Peter Sculco¹, Jason Blevins¹, Caroline Siegel¹, Dina Greenman¹, Kyriakos Kirou¹ and Susan Goodman³, ¹Hospital for Special Surgery, New York, NY, ²Columbia University VP&S, New York, NY, ³Hospital for Special Surgery, New York 10025, NY
Background/Purpose: Patients with systemic lupus erythematosus (SLE) undergoing total hip arthroplasty and total knee arthroplasty (THA and TKA) experience higher complication rates than osteoarthritis (OA)…
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)…

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