ACR Meeting Abstracts

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Abstracts tagged "Inflammasome"

  • 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: 0557 • ACR Convergence 2022

    VEXAS Syndrome Is Characterized by Blood and Tissues Inflammasome Pathway Activation and Monocyte Dysregulation

    Benjamin Terrier1, Celine Posseme2, Marie Temple3, Aurélien Corneau4, Francesco Carbone5, Eugénie Duroyon3, camille Gobeaux3, Estibaliz Lazaro6, Roderau Outh7, Guillaume Le Guenno8, Francois Lifermann9, Marie Berleur10, Thierry Weitten11, Vivien Guillotin12, Cédric Lenormand13, Hang-Korng Ea14, Mickael Ménager5, Darragh Duffy2 and Olivier Kosmider3, 1National Referral Center for Rare Systemic Autoimmune Diseases, Cochin Hospital, Paris, France, 2Institut Pasteur, Paris, France, 3Cochin Hospital, Paris, France, 4Pitié Salpêtrière Hospital, Paris, France, 5Institut Imagine, Paris, France, 6Bordeaux Hospital University, Bordeaux, France, 7CH, Perpignan, France, 8CHU, Clermont-Ferrand, France, 9Dax Hospital, Dax, France, 10CH, Versailles, France, 11CHIGAS, Gap, France, 12CHU Bordeaux, Bordeaux, France, 13CHRU, Strasbourg, France, 14Lariboisière Hospital, Paris, France

    Background/Purpose: Acquired mutations in the UBA1 gene, occurring in myeloid cells and resulting in expression of a catalytically impaired isoform of the enzyme E1, were…
  • Abstract Number: 0818 • ACR Convergence 2022

    NLRP12-associated Autoinflammatory Disease in Chinese Adult Patients: A Single-center Study

    Junke Miao and Min Shen, Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China

    Background/Purpose: NLRP12-associated autoinflammatory disease (NLRP12-AID) is an autosomal dominant autoinflammatory disorder caused by variants of the NLRP12 gene, which lead to a reduction of the…
  • Abstract Number: 1691 • ACR Convergence 2022

    Pentagalloyl Glucose Inhibits MSU-Induced Inflammation via Transforming Growth Factor-β-Activated Kinase 1 (TAK1)

    Paul Panipinto, Anil Singh, Farheen Sultan Shaikh and Salahuddin Ahmed, Washington State University, Spokane, WA

    Background/Purpose: Gout disease flares occur when primed macrophages interact with and phagocytose monosodium urate crystals (MSU), activating IL-1β via the NLRP3 inflammasome. Abrogation of inflammasome…
  • 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: 0999 • ACR Convergence 2021

    UVB-irradiated Keratinocyte-derived Extracellular Vesicles Induced Proinflammatory Responses in Macrophages

    Yubin Li1, Thomas Vazquez2, DeAnna Diaz3, Mariko Momohara4 and Victoria Werth4, 1Corporal Michael J. Crescenz VAMC, Department of Dermatology, U Penn, Philadelphia, PA, 2FIU Wertheim College of Medicine, Virginia Beach, VA, 3Philadelphia College of Medicine, Philadelphia, PA, 4University of Pennsylvania, Philadelphia, PA

    Background/Purpose: Ultraviolet B irradiation (UVB) contributes to skin inflammation. As UVB mostly affects the epidermis, with just 10% getting into the dermis, the crosstalk between…
  • Abstract Number: 1010 • ACR Convergence 2021

    Proton Pump Inhibitors Suppress IL-1 Mediated Carditis in a Murine Model of Kawasaki Disease

    Paul Tsoukas1, Melissa Kleinau2, Lysa Langevin2, Lily Morikawa3, Trang Duong1, Suzanne Tam4 and Rae Yeung5, 1Hospital for Sick Children, Toronto, ON, Canada, 2University of Toronto, Toronto, ON, Canada, 3The Hospital for Sick Children, Toronto, ON, 4Hospital for Sick Children, Toronto, ON, 5The Hospital for Sick Children, Toronto, ON, Canada

    Background/Purpose: Kawasaki disease (KD), is the leading cause of acquired heart disease in childhood. Up to 20% of patients may develop coronary artery lesions (CAL)…
  • Abstract Number: 1110 • ACR Convergence 2021

    Cryopyrin-associated Periodic Syndromes: GOSH and National Amyloidosis Centre Experience

    Ovgu Kul Cinar1, Charalampia Papadopoulou2, Amber Putland1, Karen Wynne1, Helen J Lachmann3, Despina Eleftheriou4 and Paul A. Brogan4, 1Great Ormond Street Hospital NHS Trust, London, United Kingdom, 2UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology, London, United Kingdom, 3National Amyloidosis Centre, Royal Free Campus, UCL Medical School, London, United Kingdom, 4Great Ormond Street Hospital NHS Trust, UCL GOS Institute of Child Health, Department of Infection, Immunity & Inflammation, London, United Kingdom

    Background/Purpose: CAPS is a rare, heterogenous inflammasomopathy associated with gain-of-function mutations in NLRP3 that encodes cryopyrin. Mutations in NLRP3 result in excessive IL-1ß production that…
  • Abstract Number: 1578 • ACR Convergence 2021

    NLRP3 Inflammasome Pathway Expression at Atheroma Plaques from Peripheral Artery Disease According to the Uricemic Status

    MARIANO ANDRES1, Leticia Mendieta2, Elena Argente-delcastillo1, Miguel Trigueros3 and Alberto Miñano3, 1Hospital General Universitario de Alicante-ISABIAL, Alicante, Spain, 2Universidad Miguel Hernández, San Juan de Alicante, Spain, 3Hospital General Universitario de Alicante-ISABIAL, Alicante

    Background/Purpose: Both hyperuricemia and monosodium urate crystals induce persistent inflammation. In vitro, soluble urate can prime innate immune cells and promote NLRP3 inflammasome activation and…
  • 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: 1163 • ACR Convergence 2020

    Predictors of Colchicine Response in Patients with Undefined Systemic Autoinflammatory Diseases

    Mariana Correia Marques1, Edwin Anderson1, Kathryn Williams2, Jonathan Hausmann3 and Fatma Dedeoglu4, 1Boston Children`s Hospital, Department of Pediatrics, Harvard Medical School, Boston, MA, 2Biostatistics and Research Design Center ICCTR Boston Children`s Hospital, Boston, MA, 3Boston Children’s Hospital, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, 4Boston Children's Hospital, Boston, MA

    Background/Purpose: Systemic autoinflammatory diseases (SAIDs) result from dysregulation of the innate immune system. Many patients with SAIDs have specific mutations that lead to the release…
  • Abstract Number: 1911 • ACR Convergence 2020

    IL-11 Expression in Systemic Sclerosis Pulmonary Fibroblasts Is Mediated by Caspase-1

    Caya McFalls1 and Carol Artlett1, 1Drexel University College of Medicine, Philadelphia

    Background/Purpose: Interleukin-11 (IL-11) has been shown to be associated with idiopathic pulmonary fibrosis, cardiac and kidney fibrosis. However, conflicting data shows IL-11 can be either…
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