Abstracts tagged "innate immunity"

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…
Abstract Number: 1931 • ACR Convergence 2022
NOD2 Mutations Are Associated with Upregulated Type 1 Interferon Gene Expression and Development of Granulomatous Hepatitis in Children with Autoimmune Hepatitis
Esraa Eloseily¹, Alexander Valencia², Astha Malik², Rebekah Karns², Cyd Castro Rojas², Mosab Alquraish², Rebecca Marsh³, Alexei Grom⁴ and Alexander Miethke², ¹Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Gastroenterology, Hepatology & Nutrition, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Division of Bone Marrow Transplantation & Immune Deficiency, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Mutations in the gene NOD2 encoding the nucleotide-binding oligomerization domain-containing protein 2 (NOD2) which controls innate responses to LPS have been linked to Blau…
Abstract Number: 1177 • ACR Convergence 2022
Identification of a New Type of Pro-phagocytic Macrophages in Patients with Systemic Sclerosis
Amela Hukara¹, Tracy Tabib², Michal Rudnik¹, Oliver Distler³, Przemyslaw Blyszczuk¹, Robert Lafyatis² and Gabriela Kania¹, ¹Center of Experimental Rheumatology, Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland, ²Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh, Pittsburgh, PA, ³Department of Rheumatology, University Hospital Zurich, University of Zurich, Zürich, Switzerland
Background/Purpose: Phagocytosis is a crucial cellular process, which under certain immuno-pathological conditions can be activated in macrophages in an uncontrolled manner. Biomarker studies have implicated…
Abstract Number: 1936 • ACR Convergence 2022
A De Novo Dominant-negative PSMB8 mutation is Linked to a More Severe CANDLE-like Phenotype
Sara Alehashemi¹, Adriana Almeida de Jesus², Jonas Johannes Papendorf³, Farzana Bhuyan⁴, Kat Uss⁵, Anvitha Metpally⁶, Bin Lin⁷, Sophia Park⁸, Thais C.L. Moura⁹, Renata R Dias⁹, Mayra B. Dorna⁹, Katia Kozu⁹, Adriana Sallum⁹, Lucia M. A. Campos⁹, Gijs Santen¹⁰, Jesper Kers¹⁰, Onno Teng¹¹, Karin Palmblad¹², Tom Huizinga¹⁰, Frédéric Ebstein¹³, Elke Krüger¹³ and Raphaela Goldbach-Mansky¹⁴, ¹NIH/NIAID/TADS, Clarksville, MD, ²NIAID, NIH, Silver Spring, MD, ³Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Greifswald, Germany, ⁴National Institutes of Health, Bethesda, MD, ⁵National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, ⁶NIAID, NIH, Bethesda, MD, ⁷NIH, Bethesda, MD, ⁸NIAID, Bethesda, MD, ⁹Children's Hospital of the University of São Paulo, São Paulo, Brazil, ¹⁰Leiden University Medical Center, Leiden, Netherlands, ¹¹Leiden University Medical Center, Leiderdorp, Netherlands, ¹²Karolinska University Hospital, Stockholm, Sweden, ¹³Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, ¹⁴NIH/NIAID, Potomac, MD
Background/Purpose: Homozygote (HM)/compound heterozygote (CH) and digenic (DG) mutations in proteasome subunits cause the autoinflammatory syndrome CANDLE, or proteasome-associated autoinflammatory syndrome (PRAAS). Inflammation is mediated…
Abstract Number: 1327 • ACR Convergence 2022
Immune Cell Subsets and Risk of Incident Hip Fracture in Older Adults: The Cardiovascular Health Study
Rachel Elam¹, Petra Buzkova², Joseph Delaney³, Howard Fink⁴, Joshua Barzilay⁵, Laura Carbone¹, Rick Saha¹, John Robbins⁶, Kenneth Mukamal⁷, Rodrigo Valderrabano⁸, Bruce Psaty², Russell Tracy⁹, Nels Olson⁹, Sally Huber¹⁰, Margaret Doyle⁹, Alan Landay¹¹ and Jane Cauley¹², ¹Augusta University, Augusta, GA, ²University of Washington, Seattle, WA, ³University of Manitoba, Winnipeg, MB, Canada, ⁴University of Minnesota, Minneapolis, MN, ⁵Kaiser Permanente, Atlanta, GA, ⁶UC Davis, Davis, CA, ⁷Beth Israel Deaconess Medical Center, Boston, MA, ⁸Brigham and Women's Hospital, Boston, MA, ⁹University of Vermont, Burlington, VT, ¹⁰University of Vermont, Burlingon, VT, ¹¹Rush University Medical Center, Chicago, IL, ¹²University of Pittsburgh, PIttsburgh, PA
Background/Purpose: Cell-mediated immunity may play a role in osteoporosis development. Fracture is the major consequence of osteoporosis. Thus, cell-mediated immunity may impact fracture risk. We…
Abstract Number: 2051 • ACR Convergence 2022
Targeting Janus Kinase Pathway in Sjogren’s Disease Corrects IFN-Driven Inflammation and Epithelial Dysfunction
Sarthak Gupta¹, Eiko Yamada², Hiroyuki Nakamura², Zohreh Khavandgar³, Daniel Martin², Mayank Tandon⁴, Ilias Alevizos⁵, Shyh-Ing Jang², Paola Perez², Kalie Dominick², Thomas Pranzatelli², Alan Baer⁶, john chiorini⁴ and Blake Warner³, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ²NIDCR, Bethesda, MD, ³National Institutes of Health, Bethesda, MD, ⁴NIH, Bethesda, MD, ⁵Horizon Therapeutics, Gaithersburg, MD, ⁶Johns Hopkins University School of Medicine, Baltimore, MD
Background/Purpose: Many of the inflammatory cytokines implicated in Sjogren's Disease (SjD) pathogenesis, in particular Type I and II interferons (IFNs), signal through Janus kinases (JAK)…
Abstract Number: 1565 • ACR Convergence 2022
Dissection of Ocular and Peripheral Blood Immune Phenotypes Suggests Preferential Transmigration of CD16+ Monocytes to Effector Sites in Behçet’s Disease
Yesim Ozguler¹, Ziyan Lin², Ann Cavers³, gulen Hatemi⁴, Olivier Manches⁵ and Johannes Nowatzky³, ¹Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, New York, NY, ²NYU Grossman School of Medicine, NYU Langone Applied Informatics Laboratories, New York, NY, ³NYU Grossman School of Medicine, New York, NY, ⁴Istanbul University-Cerrahpaşa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ⁵Recherche et Développement, Immunobiology and Immunotherapy in Chronic Diseases, Institute for Advanced Biosciences, Inserm U 1209, CNRS UMR 5309, Université Grenoble Alpes Etablissement Français du Sang Auvergne-Rhône-Alpes, Grenoble, France
Background/Purpose: Cellular immunity of Behçet's Disease (BD) remains poorly understood. Previous work has provided clues pointing to most innate and adaptive immune cell types in…
Abstract Number: 1663 • ACR Convergence 2022
Age-Dependent Expansion and Activation of Disease-Associated Microglia in Neuropsychiatric Symptoms of Systemic Lupus Erythematosus
Hadijat Makinde¹, Caroline Shah¹, Miranda Gurra¹, Yidan Wang¹, Deborah Winter² and Carla Cuda¹, ¹Northwestern University, Chicago, IL, ²Northwestern University, Skokie, IL
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune disease that affects multipe end organs, including the brain. Despite a prevalence of over 50% in…
Abstract Number: 0025 • ACR Convergence 2022
Wnt Pathway/Innate Immunity Equilibrium in OA Articular Cells
Eloi Franco-Trepat¹, Ana Alonso Pérez¹, María Guillán-Fresco², Miriam López Fagúndez¹, Andrés Pazos-Pérez³, Antia Crespo Golmar⁴, María Piñeiro-Ramil⁵, Ana Lois Iglesias⁶, Alberto Jorge-Mora¹ and Rodolfo Gomez¹, ¹MSP Group - Institute IDIS - CHUS - SERGAS, Santiago de Compostela, Spain, ²MSP Group - Institute IDIS - CHUS - SERGAS, Catoira, Spain, ³MSP Group - Institute IDIS - CHUS - SERGAS, Pontevedra, Spain, ⁴MSP Group - Institute IDIS - CHUS - SERGAS, León, Spain, ⁵MSP Group - Institute IDIS - CHUAC - SERGA, A Coruña, Spain, ⁶MSP Group - Institute IDIS - CHUAC - SERGAS, A Coruña, Spain
Background/Purpose: The progressive degradation of osteoarthritis (OA) cartilage has been associated with articular innate immune responses (IIR) mediated by the Toll-like receptor 4 (TLR4). Additionally,…
Abstract Number: 1690 • ACR Convergence 2022
Butyric Acid Suppresses Migration of Human Monocyte Derived Dendritc Cell by Inhibiting Actin Polymerization via mDia1 Inhibition
Takumi Nagamoto¹, takaichi okano², Yoshikazu Fujikawa³, Yuzuru Yamamoto², Hirotaka Yamada², Yo Ueda², Sho Sendo⁴ and Jun saegusa², ¹Kobe University, Kobe, Japan, ²Kobe University, Rheumatology, Kobe, Japan, ³Hyogo Prefectural Harima-Himeji General Medical Center, Himeji, Japan, ⁴University California, San Diego (UCSD), La Jolla, CA
Background/Purpose: Butyric acid is known to improve chronic inflammation such as inflammatory bowel disease and arthritis. Dendritic cells activate in inflammatory condition, migrate to regional…
Abstract Number: 0049 • ACR Convergence 2022
IL-18 Receptor-α Signaling Pathway Contributes to Autoantibody-induced Arthritis via Neutrophil Recruitment
Shinjiro Kaieda and Tomoaki Hoshino, Kurume University School of Medicine, Kurume, Japan
Background/Purpose: We have previously reported that IL-18 levels in the lungs, serum and bronchoalveolar lavage fluid (BALF) were increased in patients with idiopathic pulmonary lung…
Abstract Number: 1693 • ACR Convergence 2022
Skin Pigmentation Association with Regulation of Cyclic-GMP-AMP Following Skin Exposure to UV Light
Jie An¹, Sladjana Skopelja-Gardner², Xizhang Sun¹, Lena Tanaka¹ and Keith Elkon¹, ¹University of Washington, Seattle, WA, ²Dartmouth Hitchcock Medical Center, Lebanon, NH
Background/Purpose: Amongst the most important cytosolic DNA sensors is Cyclic GMP-AMP synthase (cGAS). Binding of DNA to cGAS results in the synthesis of the cyclic…
Abstract Number: 0148 • ACR Convergence 2022
Dysferlin Associated Autoinflammatory Disease Causing Systemic Inflammation and Sterile Lung Abcesses Through Impaired Efferocytosis
Farzana Bhuyan¹, Adriana Almeida de Jesus², Clinton Bradfield³, Mohammad Arif Rahman⁴, Andre Rastegar³, sundar ganesan³, Sachin Gaurav³, Sara Alehashemi³, Ronit Herzog⁵, Steven Holland³, Deborah Consilini⁶, Iain Fraser³ and Raphaela Goldbach-Mansky⁷, ¹National Institutes of Health, Bethesda, MD, ²NIAID, NIH, Silver Spring, MD, ³NIAID, Bethesda, MD, ⁴NCI, Bethesda, MD, ⁵Langone Medical Center, New York, NY, ⁶Nemours Children's Hospital-Delaware, Wilmington, DE, ⁷NIAID, NIH, Rockville
Background/Purpose: Using whole exome sequencing (WES), we identified a de novo mutation in DYSF encoding dysferlin in 2 unrelated patients with systemic inflammation and sterile…
Abstract Number: 1695 • ACR Convergence 2022
Dermatomyositis (DM) Macrophages Upregulate Genes Involved in the Remodeling of the Extracellular Matrix and Activate Keratinocytes in a Novel 3D Tissue Model of DM Skin Disease
Zoe Chafouleas¹, Angelique Cortez¹, James Whitley¹, Dorothea Barton¹, Lin Brown¹, Michael Whitfield², Patricia pioli³ and Sladjana Skopelja-Gardner¹, ¹Dartmouth Hitchcock Medical Center, Lebanon, NH, ²Dartmouth Geisel School of Medicine, Department of Biomedical Data Science, Lebanon, NH, ³Geisel School of Medicine at Dartmouth, Lebanon, NH
Background/Purpose: With an incidence of 10 cases per million and lack of animal or ex vivo disease models, dermatomyositis (DM) remains a debilitating disease without…
Abstract Number: 0149 • ACR Convergence 2022
Autoinflammatory Diseases Associated with NOD2 and Other Concurrent Genetic Mutations
Hafsa Nomani, Brianne Navetta-Modrov, Mark Yun and QingPing Yao, Stony Brook University, Stony Brook, NY
Background/Purpose: Systemic autoinflammatoy diseases (SAIDs) are primarily caused by abnormal innate immune response. NOD-like receptors (NLRs) are intracellular sensors to the immune process, including NOD2,…

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