Abstracts tagged "innate immunity"

Abstract Number: 1712 • ACR Convergence 2023
Identification and Functional Characterization of Eight CANDLE/PRAAS Causing Proteasome Variants in Five Unrelated Patients
Adriana Almeida de Jesus¹, Jonas J. Papendorf², Frederic Ebstein³, Sara Alehashemi⁴, Daniela Pioto⁵, Anna Kozlova⁶, Maria Teresa TErreri⁷, Anna Shcherbina⁶, Andre Rastegar⁸, Marta Rodrigues⁹, Renan Pereira¹⁰, Sophia Park¹¹, Bin Lin¹², Kat Uss¹¹, Ana Flavia da Silva Pina⁵, FLAVIO SZTAJNBOK¹³, Sofia Torreggiani¹⁴, Jennifer Stoddard¹⁵, Julie Niemela¹⁵, Sergio Rosenzweig¹⁵, Adriana Fonseca¹⁶, Marietta De Guzman¹⁷, Nicole Micheloni⁵, Melissa Fraga⁵, Sandro Perazzio¹⁸, Raphaela Goldbach-Mansky¹⁹ and Elke Krueger², ¹NIAID, NIH, Bethesda, MD, ²Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ³Nantes Université, Nantes, France, ⁴NIH/NIAID/TADS, Clarksville, MD, ⁵Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil, ⁶Center for Pediatric Hematology, Oncology, Immunology, Moscow, Russia, ⁷UNIFESP, São Paulo, Brazil, ⁸NIH, Bethesda, MD, ⁹Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil, ¹⁰Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil, ¹¹Translational Autoinflammatory Diseases Section, LCIM, NIAID, NIH, Bethesda, MD, ¹²Translational Autoinflammatory Diseases Section l LCIM, NIAID, NIH, Bethesda, MD, ¹³UFRJ/UERJ, São Paulo, Brazil, ¹⁴University of Maryland Baltimore, Baltimore, MD, ¹⁵Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, ¹⁶Instituto de Puericultura e Pediatria Martagao Gesteira / Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, ¹⁷Baylor College of Medicine, Houston, TX, ¹⁸Universidade de Sao Paulo (Unifesp); Universidade de São Paulo (USP); Fleury Laboratories, São Paulo, Brazil, ¹⁹NIH/NIAID, Potomac, MD
Background/Purpose: Mutations in genes coding for 20S proteasome subunits or proteasome assembly helpers cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or…
Abstract Number: 0796 • ACR Convergence 2023
Role of Group 2 Innate Lymphoid Cells in the Pathogenesis of Rheumatoid Arthritis
Anders Nguyen¹, Agnieszka Lastowska¹, Miriam Bollmann¹, Symeon Kourmoulakis¹, Charlotte E. van der Plas¹, Anna-Karin Hultgård Ekwall², Dietmar M. Zaiss³, Gary S Firestein⁴ and Mattias N.D Svensson⁵, ¹Department of Rheumatology and Inflammation research, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden, ²Department of Rheumatology and Inflammation research, Sahlgrenska Academy, University of Gothenburg, Kullavik, Sweden, ³Faculty of Medicine, Department of Immune Medicine, University of Regensburg, Regensburg, Germany, Regensburg, Germany, ⁴Department of Medicine, University of California San Diego, La Jolla, CA, ⁵University of Gothenburg, Gothenburg, Sweden
Background/Purpose: Immune-cell mediated activation of joint-lining fibroblast-like synoviocytes (FLS) play a key role in joint inflammation and destruction during Rheumatoid arthritis (RA). Thus, identifying factors…
Abstract Number: 1761 • ACR Convergence 2023
JAK/STAT Inhibition Modifies the Innate Lymphoid Cells 1 Immune Response in Patients with Rheumatoid Arthritis
Lidia La Barbera¹, Marianna Lo Pizzo¹, Chiara Rizzo¹, Leila Mohammadnezhad¹, federica Camarda¹, Francesco Ciccia² and Giuliana Guggino¹, ¹University of Palermo, Palermo, Italy, ²University of Campania - Luigi Vanvitelli, Naples, Italy
Background/Purpose: Recent evidence suggests that innate lymphoid cells (ILCs) might be involved in rheumatoid arthritis (RA) pathogenesis and individuals at risk of RA exhibited an…
Abstract Number: 0881 • ACR Convergence 2023
Deficiency of the Pattern-recognition Receptor CD14 Protects Against LPS-induced Inhibition of Osteoclastogenesis in Vitro
Lance Murphy¹, Kevin Burt², Baofeng Hu³, Vu Nguyen², Robert Mauck² and Carla Scanzello², ¹University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, ²University of Pennsylvania, Philadelphia, PA, ³University of Pennsylvania, Wynnewood, PA
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: 2206 • ACR Convergence 2023
Increased Frequency of Activated MAIT Cells Expressing the Gut Homing Receptor CCR9 in Patients with Radiographic Axial Spondyloarthritis
Eric Toussirot¹, Caroline Laheurte², Eléonore Gravelin², Charline Vauchy³, Marc Puyraveau³ and Philippe Saas⁴, ¹University Hospital of Besancon, Besançon, France, ²INSERM UMR1098, plateforme de biomonitoring, EFS Bourgogne-Franche-Comté, Besançon, France, ³INSERM CIC-1431, Besançon, France, ⁴INSERM UMR1098, EFS Bourgogne-Franche-Comté, Besançon, France
Background/Purpose: MAIT (mucosal associated invariant T) cells are involved in mucosa defense against bacteria. This cellular subset is characterized by a semi-invariant αβ TCR and…
Abstract Number: 0888 • ACR Convergence 2023
Functionally Selective Immunomodulator Shows Robust Efficacy in Spontaneous Lupus Mouse Model
Helene ASNAGLI¹, Simon TESSIER¹, Martyn FOSTER², Sofie DENIES³, Eef HOEBEN⁴, Joël CROUZET¹ and Annegret VAN DER AA¹, ¹Ermium Therapeutics, Paris, France, ²Experimental Pathology Consultancy, Benfleet, United Kingdom, ³SD Analytics, Bellem, Belgium, ⁴2-Bridge, Zoersel, Belgium
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex, heterogeneous autoimmune disease. There is still a high unmet need to improve current treatment options. Type 1…
Abstract Number: 2407 • ACR Convergence 2023
Immuno-permissive Antigen-presenting Cells in Giant Cell Arteritis
Shozo Ohtsuki¹, Chenyao Wang², Ryu Watanabe³, Hui Zhang³, Mitsuhiro Akiyama³, Kenneth Warrington⁴, Gerald J. Berry³, Jorg Goronzy⁴ and Cornelia M. Weyand⁵, ¹Mayo Clinic College of Medicine and Science, Stanford University School of Medicine, Rochester, MN, ²Mayo Clinic College of Medicine and Science, Rochester, MN, ³Stanford University School of Medicine, Stanford, CA, ⁴Mayo Clinic, Rochester, MN, ⁵Mayo Clinic School of Medicine and Stanford University, Rochester, MN
Background/Purpose: Giant cell arteritis (GCA) is a medium and large vessel vasculitis with pathognomonic granulomatous infiltrates in the vessel wall. During the early stages of…
Abstract Number: 0892 • ACR Convergence 2023
Single-Cell RNA Sequencing Reveals Keratinocytes and Fibroblasts Drive Hippo and TGFb Dysregulation and Fibrosis in Epidermal Overexpression of VGLL3 in Cutaneous Lupus
Mehrnaz Gharaee-Kermani¹, Allison Billi¹, Jacob Martens¹, Marisa Hildebrandt¹, Amanda Victory¹, Mitra Maz², Shannon Loftus¹, J. Michelle Kahlenberg¹ and Johann E. Gudjonsson¹, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan Medical School, Ann Arbor, MI
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast (MYOFB) accumulation, and extracellular matrix remodeling. In some subtypes of cutaneous lupus erythematosus (CLE), particularly discoid lupus…
Abstract Number: 2427 • ACR Convergence 2023
Degranulating PR3+ Myeloid Cells Characterize Proliferative Lupus Nephritis
Alessandra Ida Celia¹, Xiaoping Yang², Hana Minsky², Silvia Malvica², Michelle Petri³, the Accelerating Medicines Partnership in RA/SLE⁴, Avi Rosenberg⁵ and Andrea Fava², ¹John Hopkins University of Medicine, Rome, Italy, ²Johns Hopkins University, Baltimore, MD, ³Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Timonium, MD, ⁴Multiple, Multiple, ⁵Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: As part of the Accelerating Medicines Partnership (AMP), we discovered that urinary PR3, a neutrophil degranulation product, is associated with histological activity, indicating the…
Abstract Number: 0894 • ACR Convergence 2023
The Cellular and Spatial Type I Interferon Response Following Skin Exposure to Ultraviolet Light
Jie An¹, Xizhang Sun¹, Rayan Najjar¹, Connie Zhao¹, Paul Kong², Stephanie Weaver², Amanda Koehne², Matt Fitzgibbon² and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Fred Hutchinson Cancer Center, Seattle, WA
Background/Purpose: SLE patients characteristically have a prominent type I interferon (IFN-I) signature in lesional and non-lesional skin. We recently demonstrated that, following a single exposure…
Abstract Number: 2440 • ACR Convergence 2023
Synovial Shaping of Skin-derived Migrating Immune Cells Determines Initiation of Inflammation in Psoriatic Arthritis
Maria Gabriella Raimondo¹, Simon Rauber², Hashem Mohammadian³, Mario Angeli¹, Cong Xu¹, Aleix Rius Rigau⁴, Markus Luber¹, Hannah Labinsky⁵, Alina Ramming¹, Stefano Alivernini⁶, Jörg Distler¹, Ursula Fearon⁷, Douglas Veale⁸, Michael Sticherling⁹, Juan D Canete¹⁰, Georg Schett¹¹ and Andreas Ramming¹, ¹Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ²3 Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. 4 Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany., Erlangen, Germany, ³Department of Internal Medicine 3 – Rheumatology and Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany. Deutsches Zentrum für Immuntherapie, Friedrich Alexander University Erlangen-Nürnberg and Universitätsklinikum Erlangen, Erlangen, Germany, ⁴Department of Internal Medicine 3, Rheumatology and Clinical Immunology, Friedrich-Alexander-University Erlangen-Nürnberg (FAU) and University Hospital Erlangen, Erlangen, Germany; Deutsches Zentrum Immuntherapie (DZI), FAU Erlangen-Nürnberg and University Hospital Erlangen, Erlangen, Germany, ⁵Medical Department II, Rheumatology, University Hospital Würzburg, Würzburg, Germany, ⁶Immunology Research Core Facility, Gemelli Science and Technology Park, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy; Division of Rheumatology - Fondazione Policlinico Universitario A. Gemelli IRCCS - Università Cattolica del Sacro Cuore, Rome, Italy, ⁷Trinity College Dublin, Dublin, Ireland, ⁸St.Vincent's University Hosp, Dublin, Ireland, ⁹Friedrich-Alexander-Universität (FAU) Erlangen-Nürnberg & Universitätsklinikum Erlangen, Department of Dermatology, Erlangen, Germany, ¹⁰Hospital Clinic an IDIBAPS, Barcelona, Spain, ¹¹Friedrich-Alexander-Universität Erlangen-Nürnberg, Erlangen, Germany
Background/Purpose: Around 30% of the patients with psoriasis (PsO) develop psoriatic arthritis (PsA) overtime, suggesting the existence of a disease mediated skin-joint crosstalk. To date,…
Abstract Number: 0905 • ACR Convergence 2023
Low-Density Granulocytes and Neutrophil Extracellular Traps in Incomplete Systemic Lupus Erythematosus
Svenja Henning¹, Tobias Reimers², Berber Doornbos-van der Meer², Barbara Horvath³, Hendrika Bootsma⁴, Karina de Leeuw² and Johanna Westra², ¹University Medical Center Groningen, Department of Rheumatology and Clinical Immunology, Groningen, Netherlands, ²University Medical Center Groningen, Groningen, Netherlands, ³University Medical Center Groningen / Department of Dermatology, Groningen, Netherlands, ⁴Department of Rheumatology, University Medical Centre Groningen, Groningen, Netherlands
Background/Purpose: Incomplete SLE (iSLE) defines a group of patients with symptoms typical of SLE but not meeting sufficient criteria required for the classification of SLE.…
Abstract Number: 2442 • ACR Convergence 2023
Neutrophils Induce Contact-Dependent Expansion of Arthritogenic Th17 Cells and Are Necessary for Disease in Experimental Ankylosing Spondylitis
Hillary Struthers¹, Emily Vance¹, Kofi Asare-Konadu¹, Holly Rosenzweig² and Ruth Napier¹, ¹Oregon Health & Science University, Portland, OR, ²VA Portland Healthcare System, Portland, OR
Background/Purpose: Ankylosing spondylitis (AS) patients have irregular neutrophil responses, as indicated clinically by neutrophilia and increased neutrophil to lymphocyte ratios that positively associate with disease…
Abstract Number: 0911 • ACR Convergence 2023
Spatial Transcriptomics Reveals Normal-density and Low-density Neutrophils Are More Prevalent Than Macrophages in Lupus Nephritis Glomeruli, and Urine DNA Methylation Analyses Capture Both Myeloid Populations
Sladjana Skopelja-Gardner¹, Joshua Skydel², Alecia Roy³, Brenna Kerin³, James Whitley³, Fred Kolling⁴, Noelle Kosarek⁴, Michelle Petri⁵, Andrea Fava⁶, Lucas Salas⁷ and Christopher Burns³, ¹Department of Medicine, Dartmouth Hitchcock Medical Center, Geisel School of Medicine at Dartmouth, Lebanon, NH, ²Dartmouth-Hitchcock Medical Center, Lebanon, NH, ³Dartmouth Hitchcock Medical Center, Lebanon, NH, ⁴Geisel School of Medicine at Dartmouth, Hanover, NH, ⁵Department of Medicine, Division of Rheumatology, Johns Hopkins University School of Medicine, Timonium, MD, ⁶Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, ⁷Department of Epidemiology, Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: Despite compelling evidence that normal-density (NDN) and low-density neutrophils (LDN) are activated in the blood of lupus patients, their role in lupus nephritis (LN)…
Abstract Number: 2600 • ACR Convergence 2023
Clonal Hematopoiesis Is Associated with Giant Cell Arteritis
Background/Purpose: Giant cell arteritis (GCA) is an age-related vasculitis of large and medium vessels. Prior population studies have identified an association between GCA and hematologic…

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Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. Academic institutions, private organizations and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part a scientific presentation or presentation of additional new information that will be available at the time of the meeting) is under embargo until Saturday, November 11, 2023.

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