Abstracts tagged "innate immunity"

Abstract Number: 1662 • ACR Convergence 2023
Chronic Excess IL-18 Induces NK Deficiency, but Drives Hyperinflammation via CD8 T-cell Cytokine Overproduction and Selective Immunodeficiency
Jemy Varghese¹, Scott Canna¹, Emily Landy², Laurence Eisenlohr¹, Elise Peauroi³, Vinh Dang¹, Anastasia Frank-Kamenetskii⁴ and Jeremy Morrissette⁵, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²University of Pittsburgh, Pittsburgh, PA, ³University of Pennsylvania, Philadelphia, PA, ⁴CHOP/UPENN, Philadelphia, PA, ⁵University of Pennsylvania - Perelman School of Medicine, Philadelphia, PA
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) and Macrophage Activation Syndrome (MAS) are associated with highly elevated peripheral blood levels of the inflammasome-activated cytokine IL-18 and…
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: 1676 • ACR Convergence 2023
Tofacitinib Accelerates in Vitro Clot Formation and Delays Clot Lysis in Rheumatoid Arthritis Blood by Enhancement of Macrophage TLR4 Mediated Cytokine Responses- a New Angle on Thrombosis with JAKi in Rheumatology
Paula David¹, Thomas Macleod², Yu Shi³, Payal Ganguly¹, Cedric Duval³, Mark Harland¹, Chi Wong¹, Benazir Saleem⁴ and Dennis McGonagle⁵, ¹University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine (LIRMM), Leeds, United Kingdom, ²University of Leeds, Leeds Institute of Rheumatic and Musculoskeletal Medicine, Leeds, United Kingdom, ³Discovery and Translational Science Department, Leeds Institute of Cardiovascular and Metabolic Medicine, University of Leeds, Leeds, United Kingdom, ⁴Leeds Teaching Hospitals NHS Trust, Rheumatology, Chapel Allerton Hospital, Leeds, United Kingdom, ⁵Leeds Biomedical Research Centre, University of Leeds, Leeds, United Kingdom
Background/Purpose: Rheumatoid Arthritis (RA) patients treated with Janus Kinase inhibitors (JAKi) have reportedly higher venous thromboembolism (VTE) risk and atherosclerotic-related complications including myocardial infarction (1).Although…
Abstract Number: 0757 • ACR Convergence 2023
Identification of 23 Novel COPA Rare Exonic Non-Synonymous Variants and Their Associated Autoimmune and Inflammatory Clinical Phenotypes Among 53,364 Individuals
Pierre-Antoine Juge¹, Gregory McDermott², Keigo Hayashi², Jing Cui³, Clémence David-Gabarre⁴, Marie-Louis Frémond⁴, Holly Wobma⁵, Soumya Raychaudhuri², Elizabeth Karlson², Philippe Dieudé⁶ and Jeffrey Sparks⁷, ¹Division of Rheumatology, Inflammation, and Immunity Brigham & Women's Hospital, Boston, MA, ²Brigham and Women's Hospital, Boston, MA, ³Brigham and Women's Hospital, Boston, MA, ⁴Imagine, Paris, France, ⁵Division of Immunology, Boston Children's Hospital, Boston, MA, ⁶Assistance Publique-Hôpitaux de Paris, Bichat-Claude Bernard University Hospital, INSERM UMR1152, University de Paris Cité, Department of Rheumatology, Paris, France, ⁷Division of Rheumatology, Inflammation, and Immunity, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA
Background/Purpose: COPA syndrome is a rare, severe monogenic autoinflammatory/autoimmune disorder with variable penetrance, most commonly affecting the lungs and joints. It is caused by dominant…
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: 127 • 2023 Pediatric Rheumatology Symposium
What’s in a Name? A20 Protein Expression in an in Vitro Model of A20 Haploinsufficiency
Patricia Pontes Aires¹, DANIELA GERENT PETRY PIOTTO¹, Andre Cunha¹, Sandro Perazzio² and Maria Teresa Terreri³, ¹Universidade Federal de São Paulo, São Paulo, Brazil, ²Universidade de So Paulo; Fleury Laboratories, São Paulo, Brazil, ³UNIFESP, São Paulo, Brazil
Background/Purpose: Tumor necrosis factor alpha (TNF-alpha) induced protein 3 gene, or TNFAIP3, encodes the A20 protein, an important regulator of the NF-κB pathway. Since its…
Abstract Number: 0975 • ACR Convergence 2022
Modulation of T, B, and Innate Cell-Associated Pharmacodynamic Biomarkers in a Phase 3 Trial of Anifrolumab in Moderate to Severe SLE
Paul Newcombe¹, Richard A. Furie², Yoshiya Tanaka³, Wendy White⁴, Dominic Sinibaldi⁴, Philip Brohawn⁴, Mark Lazarus¹, Nicola Ferrari¹, Raj Tummala⁴, Hussein Al-Mossawi¹, Andre Nogueria da Costa⁵, Daniel Muthas⁵ and Madhu Ramaswamy⁴, ¹AstraZeneca, Cambridge, United Kingdom, ²Northwell Health, Great Neck, NY, ³University of Occupational and Environmental Health, Kitakyusyu Fukuoka, Japan, ⁴AstraZeneca, Gaithersburg, MD, ⁵AstraZeneca, Gothenburg, Sweden
Background/Purpose: SLE has been associated with expression of type I IFN gene signatures (IFNGS).1 Anifrolumab, a monoclonal antibody binding IFN receptor subunit 1, inhibits downstream…
Abstract Number: 1853 • ACR Convergence 2022
A Comparitive Study of NLRP3- and NLRP12-autoinflammatory Disease
Mark Yun, Brianne Navetta-Modrov, Hafsa Nomani, Jie Yang and QingPing Yao, Stony Brook University, Stony Brook, NY
Background/Purpose: NLRP3-associated autoinflammatoy disease (NLRP3-AID) and NLRP12-AID are rare autosomal dominant diseases. FACS1(familial cold autoinflammatory syndrome type 1), a subset of NLRP3-AID, is thought to…
Abstract Number: 1142 • ACR Convergence 2022
The Lupus Risk Allele Associated Xq28 Haplotype Identifies the Molecular Endotype of a Cohort of European SLE Patients with Enhanced IRAK1 Signaling
Rheyanna Clemens¹, Tyson Dawson¹, Katherine Owen², Amrie Grammer³ and Peter Lipsky¹, ¹AMPEL BioSolutions, Charlottesville, VA, ²RILITE, Crozet, VA, ³AMPEL LLC, Charlottesville, VA
Background/Purpose: Previous genetic association studies identified a 9 gene region of Xq28 containing IRAK1 as a risk locus for SLE. IRAK1 plays key roles downstream…

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