Abstracts tagged "innate immunity"

Abstract Number: 1455 • ACR Convergence 2020
Functional Characterization of PLCG2 Mutations Found in Subjects with Autoinflammation and PLCG2-Associated Antibody Deficiency and Immune Dysregulation (APLAID) Reveals Both Hypermorphic and Hypomorphic Mutants
Kathleen Baysac¹, Charles Fisher², Hiroto Nakano², Joshua Milner³ and Michael Ombrello⁴, ¹NIAMS, NIH, Bethesda, MD, ²NIAMS, NIH, Bethesda, ³Division of Allergy, Immunology and Rheumatology Columbia University Medical Center, New York, NY, ⁴Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD
Background/Purpose: PLCG2-associated antibody deficiency and immune dysregulation (PLAID) and autoinflammatory PLAID (APLAID) are autosomal dominant diseases caused by mutations of PLCG2. APLAID is clinically characterized by episodic…
Abstract Number: 0062 • ACR Convergence 2020
The Role of PGLYRP1 in the Pathogenesis of Lyme Disease
Akash Gupta¹, Gunjan Arora¹, Connor Rosen², Yongguo Cao¹, Jiri Cerny³, Carmen Booth⁴, Noah Palm², Aaron Ring² and Erol Fikrig¹, ¹Section of Infectious Diseases, Department of Internal Medicine, Yale University School of Medicine, New Haven, CT, ²Department of Immunobiology, Yale University School of Medicine, New Haven, CT, ³Faculty of Tropical AgriSciences, Czech University of Life Sciences in Prague, Prague, Czech Republic, ⁴Department of Comparative Medicine, Yale University School of Medicine, New Haven, CT
Background/Purpose: Lyme Disease is caused by the spirochete Borrelia Burgdorferi (Bb). The infection often begins in the skin, following a tick bite, and spreads to…
Abstract Number: 1517 • ACR Convergence 2020
Metabolic Regulation of Type 3 Innate Lymphoid Cells by Intestinal Bacteria-Derived Indoles in Ankylosing Spondylitis
Adam Berlinberg¹, Adam Lefferts², Emilie Regner³, Andrew Stahly⁴ and Kristine Kuhn⁴, ¹University of Colorado, Denver, CO, ²University of Colorado, Anschutz Medical Campus, Denver, CO, ³University of California San Francisco, San Francisco, CA, ⁴University of Colorado Anschutz Medical Campus, Aurora, CO
Background/Purpose: Intestinal microbial dysbiosis, intestinal inflammation, and Th17 immunity are all linked to the pathophysiology of ankylosing spondylitis (AS); however, the mechanisms linking them remain…
Abstract Number: 0063 • ACR Convergence 2020
Novel Repurposed Drugs Against Joint Inflammation Reveal Potential Use for Gout Treatment: An In Silico, In Vitro and Clinical Study
Eloi Franco-Trepat¹, Ana Alonso-Pérez¹, Maria Guillán-Fresco¹, Miriam López-Fagundez¹, Andrés Pazos-Pérez¹, Ana Lois Iglesias², Susana Belén Bravo³, Alberto Jorge-Mora¹, JJ Gómez-Reino⁴ and Rodolfo Gómez¹, ¹IDIS-CHUS - Musculoskeletal Pathology Group, Santiago de Compostela, Spain, ²IDIS-CHUS - Musculoskeletal Pathology Group, A Coruna, Spain, ³IDIS-CHUS - Proteomics Unit, Santiago de Compostela, Galicia, Spain, ⁴IDIS-CHUS - Rheumatology Group, Santiago de Compostela, Spain
Background/Purpose: Joint inflammation is a common feature across multiple rheumatic diseases. To deal with the induction of innate immune factors, targeting therapeutic targets such as…
Abstract Number: 1531 • ACR Convergence 2020
Characterization of Cytokine/chemokine Profile in Patient-derived M1/ M2 Macrophages to Identify Biomarkers for Genetically-defined Systemic Autoinflammatory Diseases
Farzana Bhuyan¹, Adriana Almeida de Jesus², Kim Johnson³, Jacob Mitchell⁴, Yan Huang⁵ and Raphaela Goldbach-Mansky⁵, ¹NIH, bhetesda, MD, ²Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, ³NIH, NIAID, Bethesda, ⁴Translational Autoinflammatory Disease Section (TADS)/NIAID/NIH, Bethesda, MD, ⁵NIH, Bethesda
Background/Purpose: Genetic mutations in key regulatory molecules of the innate immune system cause autoinflammatory diseases through propagation of hyperinflammatory responses. Monocytes/ macrophages regulate inflammatory processes…
Abstract Number: 0064 • ACR Convergence 2020
Neutrophil Extracellular Traps Are Sufficient to Activate the Alternative Pathway of Complement
Rebecca Schriefer¹, Michelle Elvington², Priyan Weerappuli³ and Alfred Kim⁴, ¹Washington University School of Medicine, Saint Louis, MO, ²Kypha, Inc., Saint Louis, MO, ³University of Michigan, Ann Arbor, MI, ⁴Washington University School of Medicine, St. Louis, MO
Background/Purpose: Systemic lupus erythematosus (SLE) replies on complement activation to drive many of the pathophysiologic features of disease. We and others have noted that SLE…
Abstract Number: 1636 • ACR Convergence 2020
8 Years Follow-Up of a Novel Autoinflammatory Disease: CD59 Malfunction Causes Hemolytic Anemia, Recurrent Guillain-Barre Syndrome, and Strokes in Pediatric Populations and Respond Well to Eculizumab and Pozelimab
Dror Mevorach¹ and Netanel Karbian¹, ¹Hadassah-University Hospital, Jerusalem, Yerushalayim, Israel
Background/Purpose: In 2013 we have described the first patients with a novel autoinflammatory disease manifested in 4 children with recurrent Guillain-Barre syndrome and hemolytic anemia…
Abstract Number: 159 • 2020 Pediatric Rheumatology Symposium
Interleukin-18 as a Key Cytokine to Understand Pathology and to Decide Appropriate Therapeutic Strategy in Chronic Arthritic Systemic Juvenile Idiopathic Arthritis
Takako Miyamae¹, Yumi Tani ¹, Manabu Kawamoto ², Takayuki Kishi ³ and Masayoshi Harigai ², ¹Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women's Medical University, Tokyo, Japan, ²Institute of Rheumatology, Tokyo Women's Medical University, TOKYO, Japan, ³Department of Pediatrics, Tokyo Women's Medical University, Tokyo, Japan
Background/Purpose: Long-term outcomes of systemic JIA are highly variable. approximately half of the patients have chronic persistent arthritis requiring extended anti-inflammatory therapy, sometimes into adulthood.…
Abstract Number: 75 • 2019 ACR/ARP Annual Meeting
Microglia-Specific Transcriptional Signatures Correlate with Behavioral Deficits in ‘Neuropsychiatric Symptoms of Systemic Lupus Erythematosus’
Hadijat Makinde¹, Elise Mike ², Chaim Putterman ³, Deborah Winter ⁴ and Carla Cuda ⁵, ¹Northwestern University, Chicago, IL, ²Albert Einstein College of Medicine, Bronx, NY, ³Albert Einstein College of Medicine, New York, NY, ⁴Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, ⁵Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, IL
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune syndrome affecting multiple organs, including the brain. More than 50% of patients experience neuropsychiatric symptoms of…
Abstract Number: 76 • 2019 ACR/ARP Annual Meeting
Effect of Ifnα and Costimulatory Blockade on Brain Infiltration in a Model of ‘Neuropsychiatric Symptoms of Systemic Lupus Erythematosus’
Hadijat Makinde¹, Chirag Raparia ², Anne Davidson ² and Carla Cuda ³, ¹Northwestern University, Chicago, IL, ²Feinstein Institutes for Medical Research, Manhasset, ³Northwestern University Feinberg School of Medicine, Division of Rheumatology, Chicago, IL
Background/Purpose: Systemic lupus erythematosus (SLE) is a complex autoimmune disease that affects many end organs including the brain. Despite a prevalence of over 50% in…
Abstract Number: 79 • 2019 ACR/ARP Annual Meeting
Unique Primed Status of Microglia Under the Systemic Autoimmune Condition of Lupus-Prone Mice
Atsushi Nomura¹, Daisuke Noto ², Goh Murayama ³, Asako Chiba ¹ and Sachiko Miyake ¹, ¹Department of Immunology, Juntendo University School of Medicine, Tokyo, Japan, ²Juntendo University School of Medicine, Tokyo, Japan, ³Department of Internal Medicine and Rhumatology, Juntendo University School of Medicine, Tokyo, Japan
Background/Purpose: Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by the production of various autoantibodies. This disease causes disabling neuropsychiatric symptoms even in the…
Abstract Number: 800 • 2019 ACR/ARP Annual Meeting
Cryopyrin-Associated Periodic Syndrome in Korea: 19 Years of Experience
Young Ho Kim¹, Bongjik Kim ², Byung Yoon Choi ², Haeng Jin Lee ³, Dae-Chul Jeong ⁴, Jinhee Han ², Hye-Rim Park ², Jayoung Oh ², Seungmin Lee ², Dooyi Oh ⁵ and Soyoung Lee ¹, ¹Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ²Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seongnam, Kyonggi-do, Republic of Korea, ³Department of Ophthalmology, Seoul National University Hospital, Seoul, Republic of Korea, ⁴Department of Pediatrics, The Catholic University of Korea, Seoul, Seoul-t'ukpyolsi, Republic of Korea, ⁵Department of Otorhinolaryngology-Head and Neck Surgery, Seoul National University Bundang Hospital, Seoul, Kyonggi-do, Republic of Korea
Background/Purpose: Cryopyrin-associated periodic syndrome (CAPS) is rare auto-inflammatory disorder characterized by recurrent episodes fever with variable manifestation of systemic inflammation such as urticarial skin rash,…
Abstract Number: 1020 • 2019 ACR/ARP Annual Meeting
NLRP12 Regulates Interferon-α Expression and Is a Biomarker for Disease Activity of Systemic Lupus Erythematosus
Ming-Han Chen¹, Yen-Po Tsao ¹ and Szu-Ting Chen ², ¹Division of Allergy, Immunology & Rheumatology/Taipei Veterans General Hospital, Taipei, Taiwan (Republic of China), ²Institutes of Clinical Medicine, National Yang-Ming University, Taipei, Taiwan (Republic of China)
Background/Purpose: Systemic lupus erythematous (SLE) is a systemic autoimmune disease with diverse etiological factors. It was well recognized that interferon (IFN) signature did the perpetration…
Abstract Number: 2736 • 2019 ACR/ARP Annual Meeting
Mucosal-associated Invariant T Cells Can Be Therapeutically Targeted in Lupus
Goh Murayama¹, Asako Chiba ², Tomohiro Mizuno ³, Atsushi Nomura ², Taiga Kuga ⁴, HIrofumi Amano ⁴, Ken Yamaji ⁴, Naoto Tamura ⁴ and Sachiko Miyake ², ¹Department of Internal Medicine and Rhumatology, Juntendo University School of Medicine, Tokyo, Japan, ²Department of Immunology, Juntendo University School of Medicine, Tokyo, Japan, ³Department of Immunlogy, Juntendo University School of Medicine, Tokyo, Japan, ⁴Department of Internal Medicine and Rheumatology, Juntendo University School of Medicine, Tokyo, Japan
Background/Purpose: Mucosal-associated invariant T (MAIT) cells are innate T cells that are restricted by the nonpolymorphic MHC-related molecule-1 (MR1) and express a semi-invariant TCRα chain:…
Abstract Number: 2807 • 2019 ACR/ARP Annual Meeting
Activation of the STING Pathway Is a Shared Feature of Salivary Gland and Lung Inflammation in Sjogren’s Syndrome
Joanna Papinska ¹, Grzegorz Gmyrek ², Umesh Deshmukh¹ and Harini Bagavant ¹, ¹Oklahoma Medical Research Foundation, Oklahoma City, OK, ²Oklahoma Medical Research Foundation, Oklahoma City
Background/Purpose: Sjögren’s syndrome is a chronic autoimmune disorder characterized by an increased type 1 interferon gene signature. The engagement of stimulator of interferon genes (STING)…