Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 0891 • ACR Convergence 2023
Selective and Potent Inhibition of Cyclic GMP-AMP Synthase (cGAS) Fully Normalizes Autoinflammation Across Tissues in a Trex1-/- Mouse Model for Type I Interferonopathies
Arnaud Bourin¹, Zuzana Hořejší², Isabelle Cambré¹, Juraj Dobiaš², Mikhail Klychnikov², Radek Liboska², Maroš Smolíček², Zdeněk Vavřina², Wanda Haeck¹, Hugo Klaassen¹, Marnik Nijs¹, Kristine Metzger¹, Sandro Boland¹, Dries De Clercq¹, Sara Allasia¹, Gunter Carlens¹, Patrick Chaltin³, Arnaud Marchand¹, Ondřej Páv², Matthias Versele¹ and Gabriel Birkuš², ¹Centre for Drug Design and Discovery (CD3) - Cistim, Leuven, Belgium, ²Institute of Organic Chemistry and Biochemistry, Prague, Czech Republic, ³Centre for Drug Design and Discovery (CD3) - KU Leuven, Leuven, Belgium
Background/Purpose: The cGAS enzyme is a sensor of cytosolic double-strand (ds) DNA. It serves to detect viruses and elicits an acute, transient cGAMP-STING mediated type…
Abstract Number: 1775 • ACR Convergence 2023
The Peptidyl Arginine Deiminase Inhibitor BB-CLA Decreases the Inflammatory and Fibrotic Responses in Macrophages and Rheumatoid Arthritis Synovial Fibroblasts Exposed to Fibrinogen Modified with Malondialdehyde-Acetaldehyde Adduct and Citrulline
Jack Mordeson¹, Nozima Aripova¹, Michael Duryee¹, James O'Dell¹, Bryant England¹, Daniel Anderson¹, Ted R Mikuls² and Geoffrey Thiele¹, ¹University of Nebraska Medical Center, Omaha, NE, ²Division of Rheumatology and Immunology, University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Peptide citrullination and adduction with malondialdehyde-acetaldehyde adduct (MAA) are post-translational modifications involved in the pathogenesis of rheumatoid arthritis (RA). Anti-cyclic-citrullinated peptide antibodies are >90%…
Abstract Number: 2053 • ACR Convergence 2023
Predictive Factors Associated with Treatment Response in Chronic Nonbacterial Osteomyelitis
Katherine Nowicki¹, Nathan Rogers², Carson Keeter², Nathan Donaldson², Jennifer Soep² and Yongdong (Dan) Zhao³, ¹University of Colorado, Lakewood, CO, ²University of Colorado, Aurora, CO, ³University of Washington, Redmond, WA
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO) is characterized by sterile inflammatory bone lesions and most commonly affects skeletally immature children. Non-steroidal anti-inflammatory drugs (NSAIDs) are the…
Abstract Number: 0255 • ACR Convergence 2023
Lymphoid Mass in the Inferior Turbinate with IgG4 Producing Cells: A Nasal Manifestation of IgG4-RD or a Distinct Chronic Inflammatory Disease?
Ji In Jung¹, Ju Yeon Kim², Jun Won Park², Eun Bong Lee², Jin Kyun Park³ and Doo Hee Han², ¹Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital, Seoul, South Korea, ²Seoul National University Hospital, Seoul, South Korea, ³Seoul National University College of Medicine, Seoul, South Korea
Background/Purpose: IgG4-related disease (IgG4-RD) is a rare, multi-system, fibro-inflammatory disorder characterized by the infiltration of lymphoplasmacytic cells, storiform fibrosis, obliterative phlebitis, and the presence of…
Abstract Number: 1093 • ACR Convergence 2023
Development of Adaptive Immunity Against Different SARS-CoV-2 Variants over the Course of Three COVID-19 Vaccinations in Patients with Inflammatory Rheumatic Diseases
Liam Huppke¹, Paul Wratil², Marie Bischof², Stefan Wolfrum¹, Fabian Ullrich¹, Delila Singh¹, Julia Lichtnekert¹, Lea Grümme¹, Christina Gebhardt¹, Klaus Krüger³, Franziska Wiesent⁴, Alla Skapenko¹, Oliver Keppler⁵ and Hendrik Schulze-Koops⁶, ¹Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, University of Munich, Munich, Germany, ²Max von Pettenkofer Institute & Gene Centre, Virology, National Reference Centre for Retroviruses, LMU Munich, Munich, Germany, ³Praxiszentrum St. Bonifatius, Munich, Germany, ⁴Endokrinologikum München, Munich, Germany, ⁵Max von Pettenkofer Institute & Gene Centre, Virology, National Reference Centre for Retroviruses, LMU München, Munich, Germany, ⁶Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, Ludwig-Maximilians–University Munich, Munich, Germany
Background/Purpose: Even though individuals with inflammatory rheumatic diseases (IRDs) were excluded from the SARS-CoV-2 vaccine trails, studies have shown that these individuals at risk of…
Abstract Number: 1817 • ACR Convergence 2023
Geographic Variability of Inflammatory Bowel Disease Related Mortality, 1999-2019; CDC WONDER
Saleha Aziz¹, Anum Akhlaq¹, Sana Gurz² and Yousaf Zafar¹, ¹University of Mississippi Medical Center, Jackson, MS, ²University of Nevada, Las Vegas, NV
Background/Purpose: The United States makes a significant contribution to Inflammatory Bowel Disease (IBD) related disease burden worldwide. However, data on geographic variability of IBD related…
Abstract Number: 2058 • ACR Convergence 2023
IL-1 Blocking Treatment Slows the Progression of Sensorineural Hearing Loss in Patients with NOMID
Sara Alehashemi¹, Ana M. Ortega-Villa², Megha Garg³, Katherine Myint-Hpu⁴, Kim Johnson⁵, FARZANA BHUYAN⁶, Kelly King⁷, Chris Zalewski⁷, Danielle Fink⁸, Douglas B. Kuhns⁸, John Butman⁹, Carmen Brewer⁷, H. Jeffrey Kim⁷, Dean Follmann² and Raphaela Goldbach-Mansky¹⁰, ¹NIH/NIAID/TADS, Clarksville, MD, ²Biostatistics Research Branch, Division of Clinical Research, NIH, Bethesda, MD, ³Rochester Regional Health, Pittsford, NY, ⁴NIAID, NIH, Bethesda, MD, ⁵NIH, NIAID, Bethesda, MD, ⁶National Institutes of Health, Bethesda, MD, ⁷National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD, ⁸Frederick National Laboratory for Cancer Research, Bethesda, MD, ⁹Clinical Center, NIH, Bethesda, MD, ¹⁰NIH/NIAID, Potomac, MD
Background/Purpose: NOMID is a severe form of cryopyrin-associated periodic syndrome characterized by systemic inflammation and CNS manifestations, including sensorineural hearing loss. IL-1 blocking agents, Anakinra…
Abstract Number: 0258 • ACR Convergence 2023
Understanding Monogenic Behçet’s Disease Pathophysiology: Impact of Pathogenic Variant L227X Associated with Autoinflammatory A20 Haploinsufficiency on Cellular Survival and Proliferation
Patricia Aires¹, Daniela Pioto¹, Maria Teresa TErreri² and Sandro Perazzio³, ¹Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil, ²UNIFESP, São Paulo, Brazil, ³Universidade de Sao Paulo (Unifesp); Universidade de São Paulo (USP); Fleury Laboratories, São Paulo, Brazil
Background/Purpose: A20, encoded by TNFAIP3, plays a critical role in NF-κB pathway regulation. A20 haploinsufficiency is a monogenic disorder form of Behçet's disease with a…
Abstract Number: 1106 • ACR Convergence 2023
Monosodium Urate and Calcium Pyrophosphate Crystal-induced Inflammation Relies on Cell Volume Regulation and LRRC8/VRAC Channel Activation
Twinu Wilson Chirayath¹, mete kayatekin², Isabelle Rubera³, Nghia Pham⁴, FREDERIC LIOTE⁴, Pascal Richette⁵, Vincent COMPAN⁶, François Rassendren⁷, Christophe Duranton³ and Hang Korng EA⁴, ¹INSERM, Paris, France, ²CNRS-UMR7370, Nice, France, ³CNRS-UMR 7370, Nice, France, ⁴INSERM-BIOSCAR, Paris, France, ⁵Lariboisière Hospital, Paris, France, ⁶CNRS-IGF, Montpellier, France, ⁷CNRS UMR 5203, Montpellier, France
Background/Purpose: Monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals are responsible for interleukin (IL)-1β dependent acute arthritis. The release of mature IL-1β is dependent on…
Abstract Number: 1893 • ACR Convergence 2023
Abnormalities Detected with [18F]-FDG-PET/CT Imaging in VEXAS Syndrome
Albrecht Betrains¹, Vincent Jachiet², Yannick Dieudonne³, Jérémie Dion⁴, Estibaliz Lazaro⁵, Claire De Moreuil⁶, Samuel Ardois⁷, Sylvie Grosleron⁸, Jean-benoit Arlet⁹, Cécile-Audrey Durel¹⁰, Laure Delaval¹¹, Sylvain Audia¹², Cécile Golden¹³, Barbara Nicolas¹³, Vincent Langlois¹⁴, Antoinette Perlat⁷, Frédéric Vandergheynst¹⁵, Thomas Moulinet¹⁶, Maxime Samson¹⁷, Daniel Blockmans¹, Olivier Kosmider¹⁸, Sophie Georgin-Lavialle¹⁹, Arsène Mekinian²⁰ and Benjamin Terrier²¹, ¹Department of General Internal Medicine, University Hospitals Leuven, Department of Microbiology, Immunology, and Transplantation, KU Leuven, Leuven, Belgium, ²Service de médecine interne et Inflammation-Immunopathology-Biotherapy Department (DMU i3), Sorbonne Université, AP-HP, Hôpital Saint Antoine, Paris, France, ³Department of Clinical Immunology and Internal Medicine, National Reference Centre for Systemic Autoimmune Diseases (CNR RESO), Strasbourg University Hospital, Strasbourg, France, ⁴Internal Medicine Department, Toulouse University Hospital, Toulouse, France, ⁵Bordeaux Hospital University, Pessac, France, ⁶CHU de Brest, Brest, France, ⁷CHU Rennes, Rennes, France, ⁸CH Agen-Nérac, Agen, France, ⁹Hôpital Georges-Pompidou APHP, Paris, France, ¹⁰CHU Lyon, Lyon, France, ¹¹Hôpital Bichat APHP, Paris, France, ¹²Department of Internal Medicine and Clinical Immunology, Dijon-Bourgogne University Hospital, Dijon, France, ¹³CHU Dijon, Dijon, France, ¹⁴Service de Médecine Interne, Hôpital Jacques Monod, Le Havre, France, ¹⁵Université Libre de Bruxelles, Bruxelles, Belgium, ¹⁶Department of Internal Medicine, Centre hospitalier universitaire de Nancy, Nancy, France, ¹⁷Department of Internal Medicine and Clinical Immunology, Dijon University Hospital, Dijon, France, ¹⁸Hôpital Cochin APHP, Paris, France, ¹⁹AP-HP, Tenon hospital, Paris, France, ²⁰Department of Internal Medicine, Hôpital Saint-Antoine, AP-HP, Paris, France, ²¹Department of Internal Medicine, Hôpital Cochin, AP-HP, Paris, France
Background/Purpose: VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is anautoinflammatory syndrome caused by somatic mosaicism in the UBA1 gene. [18F]-FDG-PET/CT is often performed during…
Abstract Number: 2059 • ACR Convergence 2023
Management of Iatrogenic, Recombinant Interleukin-1 Receptor Antagonist-type Amyloidosis on NOMID in Patients on Anakinra
Sara Alehashemi¹, Anvitha Metpally², Surendra Dasari³, Kat Uss², Londa Hathaway⁴, Douglas B. Kuhns⁵, Danielle Fink⁵, Chyi-Chia Richard Lee⁶, Leslie A Castelo-Soccio⁴, Edward W. Cowen⁴, Samih H. Nasr⁷, Ellen McPhail⁷ and Raphaela Goldbach-Mansky⁸, ¹NIH/NIAID/TADS, Clarksville, MD, ²NIAID, NIH, Bethesda, MD, ³Department of Quantitative Health Sciences, Mayo Clinic, Rochester, MN, ⁴NIAMS, NIH, Bethesda, MD, ⁵Frederick National Laboratory for Cancer Research, Bethesda, MD, ⁶NCI, NIH, Bethesda, MD, ⁷Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, ⁸NIH/NIAID, Potomac, MD
Background/Purpose: Patients with Neonatal-Onset Multisystem Inflammatory Disease (NOMID) often require long-term high-dose treatment with anakinra at 5-8 mg/kg daily subcutaneous injections to control CNS inflammation…
Abstract Number: 0259 • ACR Convergence 2023
Preliminary Experience with a Novel “Fix” for Deep Epitope and Transcriptional Phenotyping of Fragile Cells from Autoinflammatory Flares
Hallie Carol¹, Emily Landy² and Scott Canna¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²University of Pittsburgh, Pittsburgh, PA
Background/Purpose: Autoinflammatory diseases (AID) are characterized by inflammation and immunopathology due to primary defects in the innate immune response. Neutrophils (PMN) feature prominently in the…
Abstract Number: 1125 • ACR Convergence 2023
A Rheumatologic Clinical Profile of the VEXAS Syndrome: Results from a Survey Conducted Among Rheumatologic Units of 126 Hospitals Across Spain
Marta López I Gómez¹, Paula García Escudero², Marta López³, Berta Magallanes López⁴, Meritxell Salles Lizarzaburu⁵, Beatriz Frade Sosa⁶, Elena Riera⁷, Ernesto Trallero Araguás⁸, MARIA ELVIRA DIEZ ALVAREZ⁹, Francisco Javier Toyos Sáenz de Miera¹⁰, Alicia Garcia Dorta¹¹, José Ángel Hernández Beriain¹², Marta Ibáñez Martínez¹³, carolina Merino¹⁴, PALOMA VELA¹⁵, Ana Victoria Orenes Vera¹⁶, Diego Dios Santos¹⁷, Jose Alberto Miranda Filloy¹⁸, Clara García Belando¹⁹, María Rodríguez Laguna²⁰, Ignacio Vázquez²¹, Joaquin Belzunegui²², Judit Font²³, Carlos de Miguel Sánchez²⁴, Zuriñe Ortiz de Zárate Caballero²⁵ and Jaime Calvo Alén²⁶, ¹Hospital Universitario de Araba, Pamplona, Spain, ²Hospital Universitario Araba, Bilbao, Spain, ³Hospital Universitario Arava, Pamplona, Spain, ⁴Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, ⁵C. H. de Manresa - Fundació Althaia, Barcelona, Spain, ⁶Hospital Clínic de Barcelona, Barcelona, Spain, ⁷H. U. Mútua Terrasa, Barcelona, Spain, ⁸Department of Rheumatology, Hospital Universitario Vall d'Hebron, Barcelona, Spain, ⁹Rheumatology, Hospital Universitario de León, León, Spain, ¹⁰Hospital Virgen Macarena, Sevilla, Spain, ¹¹Rheumatology Unit, Hospital Universitario de Canarias, Santa Cruz de Tenerife, Spain, ¹²Rheumatology, Hospital Insular de Gran Canaria, Las Palmas de Gran Canaria, Spain, ¹³C. A. U. de Salamanca, Salamanca, Spain, ¹⁴Rheumatology Department Hospital Universitario Puerta de Hierro, Majadahonda (Madrid), Spain, ¹⁵Rheumatology, Hospital General Universitario Alicante, Alicante, Spain, ¹⁶H. Arnau de Vilanova, Valencia, Spain, ¹⁷Rheumatology department, Complexo Hospitalario Universitario A Coruña (CHUAC). Instituto de Investigación Biomédica A Coruña (INIBIC), A Coruña, Spain, ¹⁸C. H. U. Lucus Augusti, Lugo, Spain, ¹⁹Hospital Los Arcos del Mar Menor, Murcia, Spain, ²⁰H. Clínico San Carlos, Madrid, Spain, ²¹H. U. Dr. Peset, Valencia, Spain, ²²H. C. Alto Deba, Donostia, Spain, ²³Hospital Universitario Germans Trias i Pujol, Badalona, Spain, ²⁴Hospital Universitario de Araba, Hematology, VItoria, Spain, ²⁵Hospital Universitario de Araba, Internal Medicine, Vitoria, Spain, ²⁶Hospital Universitario Araba, Vitoria, Spain
Background/Purpose: The newly described VEXAS syndrome is a very heterogenous disease with rheumatologic and hematologic manifestations, caused by somatic mutations affecting UBA1 gene, that still…
Abstract Number: 1913 • ACR Convergence 2023
Neurosarcoidosis Disease Epidemiology. University Hospital in Northern Spain 1999-2019
Ana Serrano-Combarro¹, Alba Herrero-Morant², Lara Sanchez-Bilbao³, Inigo Gonzalez-Mazon³, David Martinez-Lopez⁴, Jose Luis Martin-Varillas⁵, Raul Fernandez-Ramon⁶ and Ricardo Blanco⁷, ¹Hospital Universitario Marques de Valdecilla, IDIVAL, Santander, Spain, ²Hospital Universitario Marqués de Valdecilla, Ontinyent, Spain, ³Hospital Universitario Marques de Valdecilla, Santander, Spain, ⁴Hospital de Sierrallana, Santander, Spain, ⁵Hospital de Laredo, Laredo, Spain, ⁶Hospital Universitario Marqués de Valdecilla, Santander, Spain, ⁷Hospital Universitario Marqués de Valdecilla, IDIVAL, Santander, Spain
Background/Purpose: Neurosarcoidosis (NS) is a serious and relative uncommon complication of sarcoidosis. Data on incidence is scarce and varies worldwide. Our objective is toestimate NS…
Abstract Number: 2175 • ACR Convergence 2023
Real-World Drug Persistence of GP2015, an Etanercept Biosimilar, in Patients with Rheumatoid Arthritis: Results from the Multi-Country COMPACT Study
Marc Schmalzing¹, Charlotte Both², Ines Brueckmann², Javier de Toro Santos³, Tom Sheeran⁴, Herbert Kellner⁵ and Ayman Askari⁶, ¹University Hospital, Rheumatology/Clinical Immunology, Department of Internal Medicine II, Würzburg, Germany, ²Sandoz Hexal AG, Rheumatology, Holzkirchen, Germany, ³University Hospital Coruña, Rheumatology, A Coruña, Spain, ⁴University of Wolverhampton, New Cross Hospital, Wolverhampton, United Kingdom, ⁵Hospital Neuwittelsbach, Center for Rheumatology and Gastroenterology, Munich, Germany, ⁶Robert Jones and Agnes Hunt Orthopaedic Hospital NHS Foundation Trust, Rheumatology, Shropshire, United Kingdom
Background/Purpose: COMPACT is a multi-country, non-interventional study that evaluated the drug persistence, effectiveness, safety and patient-reported outcomes in patients with rheumatoid arthritis (RA), axial spondyloarthritis…

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