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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