Abstracts tagged "Inflammasome"

Abstract Number: 1108 • ACR Convergence 2024
The Needed Daily Dose of Colchicine in Patients with Familial Mediterranean Fever May Be Higher in Women, a Study on Behalf of the JIR Cohort
Ilenia Di Cola¹, Alessandra Bartoli², Lea Savey³, Fatima Bensalek⁴, Marion Delplanque⁴, Rim bourguiba⁴, Zohra Aknouche⁴, Isabelle Kone Paut⁵, Linda Rossi Semerano⁵, Isabelle Melki⁶, Brigitte Bader-Meunier⁷, Bénédicte Neven⁶, PIERRE QUARTIER⁸, Guilaine Boursier⁹, Irina Giurgea¹⁰, Laurence Cuisset¹¹, Gilles Grateau⁴, Veronique Hentgen¹² and Sophie Georgin-lavialle¹³, ¹University of L'Aquila, L'Aquila, Italy, ²Internal Medicine Department, Tenon Hospital, AP-HP, Paris, France, italy, Italy, ³Internal Medicine Department, Tenon Hospital, AP-HP, Paris, France, france, France, ⁴Internal Medicine Department, Tenon Hospital, AP-HP, Paris, France, Paris, France, ⁵Kremlin Bicêtre hospital, Le Kremlin Bicêtre, France, ⁶Necker Hospital, Paris, France, ⁷Necker Hospital, Assistance Publique Hopitaux de Paris, Paris, France, ⁸Université Paris-Cite, IMAGINE Institute, Necker Children’s Hospital, Paris Cedex 15, France, ⁹University of Montpellier, Montpellier, ¹⁰Trousseau hospital, Paris, France, ¹¹Cochin hospital, Paris, France, ¹²Laboratoire de Génétique des Maladies Rares et Autoinflammatoires, Département de Génétique Médicale, Maladies Rares et Médecine Personnalisée, CEREMAIA, CHU de Montpellier, Univ Montpellier, Montpellier, France, Le Chesnay, France, ¹³Sorbonne Université, Department of internal medicine, Tenon Hospital, Assistance Publique-Hôpitaux de Paris (AP-HP), Paris, France
Background/Purpose: Familial Mediterranean Fever (FMF) is the most common autoinflammatory monogenic disease worldwide, and it is associated with mutations in MEFV gene. Colchicine is the…
Abstract Number: 2046 • ACR Convergence 2024
Duration of Disease and Long-term Outcomes in Patients with Difficult-to-treat Recurrent Pericarditis: A Chronic Condition Treated with NSAIDs, Colchicine, Corticosteroids, and Anti-IL-1 Agents
Antonio Brucato¹, Elisa Ceriani², Francesco Agozzino³, Silvia Berra⁴, Antonio Gidaro², Silvia Macchi⁵, Letizia Vena⁵, Paolo Bindi⁵, Alberto Pavarani⁶, Francesco Moda⁷, Ludovico Luca Sicignano⁸, Celeste Murace⁸, Elena Verrecchia⁸, Caterina Chiara Decarlini⁹, Silvia Maestroni¹⁰, Gabriella Marinaro⁴, Lucia Trotta³, Massimo Pancrazi³, Lisa Serati³, Enrica Negro³, Claudia Gabiati³, Elisa Calabrò³, Angela Mauro¹¹, Luisa Carrozzo³, Emanuele Bizzi³ and Massimo Imazio¹², ¹Department of Biomedical and Clinical Sciences, University of Milano, Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milano, Lombardia, Italy, ²Department of Internal Medicine, Ospedale Luigi Sacco, Milano, Milan, Italy, ³Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milan, Italy, ⁴Department of Internal Medicine, Ospedale Fatebeneratelli, Milano, Milano, Italy, ⁵University of Milano, Milan, Italy, ⁶University of Milano, Milan, ⁷Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy, ⁸Fondazione Policlinico Universitario "A. Gemelli" IRCCS, Rome, Rome, Italy, ⁹Cardiology Division, Cardiovascular Department, San L. Mandic Hospital, Merate, Lecco, Italy, ¹⁰Internal Medicine Department, Papa Giovanni XXIII Hospital, Bergamo, Bergamo, Italy, ¹¹Pediatric Rheumatology Unit, Department of Childhood and Developmental Medicine, Fatebenefratelli-Sacco Hospital, Milan, Italy, ¹²University of Udine, and Cardiothoracic Department, University Hospital Santa Maria della Misericordia, Udine, Milan, Italy
Background/Purpose: Recurrent pericarditis (RP) challenges pts and clinicians in terms of long-term management. Objective of this study was to evaluate the remission rate (drug-free for…
Abstract Number: 2201 • ACR Convergence 2024
Immunodeficiency-Related Monogenic Autoinflammatory Diseases; Expanding Spectrum of Immunodysregulation Disorders
Alhanouf Alsaleem¹, Lujain Akbar² and Sulaiman Al-Mayouf³, ¹KFSH&RC, Riyadh, Saudi Arabia, ²RIYADH, RIYADH, Saudi Arabia, ³KFSHRRC, Riyadh, Saudi Arabia
Background/Purpose: Human inborn errors of immunity are caused by monogenic germline mutations characterized by Immunodeficiency like features with increase susceptibility to infection as well as…
Abstract Number: 2518 • ACR Convergence 2024
Behçet’s Disease Immune Landscape Exhibits a Universal NF-kB-mediated Hyperactivation Pattern with Cell-specific TNFAIP3 Responses and a Superimposed IFN-regulated Endotype
Yesim Ozguler¹, Olivier Manches², Didar Ucar³, Ziyan Lin², Alireza Khodadadi-Jamayran², Aristotelis Tsirigos², Gulen Hatemi¹ and Johannes Nowatzky⁴, ¹Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ²NYU Grossman School of Medicine, New York, NY, ³Istanbul University-Cerrahpasa, Cerrahpasa Medical Faculty, Department of Ophthalmology, Istanbul, Turkey, ⁴New York University Grossman School of Medicine, New York, NY
Background/Purpose: The immune landscape of Behçet’s disease (BD) remains ill-defined, lacking a unifying picture, precise disease phenotype-endotype correlations, and an understanding of immunity at target…
Abstract Number: 0058 • ACR Convergence 2023
Macrophage Extracellular Traps Induced by Monosodium Urate or Calcium Pyrophosphate Crystals Form Independently of NLRP3 Inflammasome Activation
Douglas Daoudlarian¹, André Tiaden², Stavros Giaglis³, Ulrich Walker⁴, Petr Broz⁵, Tobias Maningold⁶ and Diego Kyburz¹, ¹University Hospital Basel, Basel, Switzerland, ²Laboratory for Experimental Rheumatology, Department of Biomedicine, University of Basel, Basel, Switzerland, ³University of Basel/University Hospital of Basel, Basel, Switzerland, ⁴Basel University Hospital, Basel, Switzerland, ⁵University of Lausanne, Lausanne, Switzerland, ⁶Department of Rheumatology, University Hospital Bern (Inselspital), University Bern, Bern, Switzerland
Background/Purpose: Monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals are potent inducers of inflammation by activation of the inflammasome in neutrophils phagocytosing the crystals and…
Abstract Number: 1106 • ACR Convergence 2023
Monosodium Urate and Calcium Pyrophosphate Crystal-induced Inflammation Relies on Cell Volume Regulation and LRRC8/VRAC Channel Activation
Twinu Wilson Chirayath¹, mete kayatekin², Isabelle Rubera³, Nghia Pham⁴, FREDERIC LIOTE⁴, Pascal Richette⁵, Vincent COMPAN⁶, François Rassendren⁷, Christophe Duranton³ and Hang Korng EA⁴, ¹INSERM, Paris, France, ²CNRS-UMR7370, Nice, France, ³CNRS-UMR 7370, Nice, France, ⁴INSERM-BIOSCAR, Paris, France, ⁵Lariboisière Hospital, Paris, France, ⁶CNRS-IGF, Montpellier, France, ⁷CNRS UMR 5203, Montpellier, France
Background/Purpose: Monosodium urate (MSU) and calcium pyrophosphate (CPP) crystals are responsible for interleukin (IL)-1β dependent acute arthritis. The release of mature IL-1β is dependent on…
Abstract Number: 2058 • ACR Convergence 2023
IL-1 Blocking Treatment Slows the Progression of Sensorineural Hearing Loss in Patients with NOMID
Sara Alehashemi¹, Ana M. Ortega-Villa², Megha Garg³, Katherine Myint-Hpu⁴, Kim Johnson⁵, FARZANA BHUYAN⁶, Kelly King⁷, Chris Zalewski⁷, Danielle Fink⁸, Douglas B. Kuhns⁸, John Butman⁹, Carmen Brewer⁷, H. Jeffrey Kim⁷, Dean Follmann² and Raphaela Goldbach-Mansky¹⁰, ¹NIH/NIAID/TADS, Clarksville, MD, ²Biostatistics Research Branch, Division of Clinical Research, NIH, Bethesda, MD, ³Rochester Regional Health, Pittsford, NY, ⁴NIAID, NIH, Bethesda, MD, ⁵NIH, NIAID, Bethesda, MD, ⁶National Institutes of Health, Bethesda, MD, ⁷National Institute on Deafness and Other Communication Disorders, NIH, Bethesda, MD, ⁸Frederick National Laboratory for Cancer Research, Bethesda, MD, ⁹Clinical Center, NIH, Bethesda, MD, ¹⁰NIH/NIAID, Potomac, MD
Background/Purpose: NOMID is a severe form of cryopyrin-associated periodic syndrome characterized by systemic inflammation and CNS manifestations, including sensorineural hearing loss. IL-1 blocking agents, Anakinra…
Abstract Number: PP11 • ACR Convergence 2023
We Suffered For Decades, But Then She Was Born
Ian Stedman¹ and Barbara Stedman², ¹Canadian Autoinflammatory Network, Vaughan, ON, Canada, ²Canadian Autoinflammatory Network, Turkey Point, ON, Canada
Background/Purpose: I was born in 1981; my mother in 1949; and my grandmother in 1926. Our story runs at least four generations deep, that we…
Abstract Number: 032 • 2023 Pediatric Rheumatology Symposium
From Bedside to Bench and Back: Discovery of a Novel Missense Variant in NLRP3 Causing Atypical Cryopyrin-Associated Periodic Syndromes with Hearing Loss as the Primary Presentation, Responsive to Anti-IL1 Therapy
Merav Birk-Bachar¹, Hadar Cohen², Yoel Levinsky³, rotem tal⁴, Gil Amarilyo⁵, Meirav Sokolov⁶, Efrat Sofrin-Drucker⁷, Naama Orenstein⁷, Gabriel Lidzbarsky⁷, Liora Kornreich⁸, Eyal Raveh⁶, Nesya Kropach-Gilad⁷, Motti Gerlic² and Liora Harel⁹, ¹Pediatric Rheumatology Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ²Sackler Faculty of Medicine, Immunology Department, Tel Aviv University, Tel Aviv, Israel, ³Schneider Children's Medical Center of Israel, Tel Aviv University, Petach Tikva, Israel, ⁴Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁵Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁶Pediatric Ear Nose and Throat Unit Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁷Recanati Genetic Institute, Rabin Medical Center-Beilinson Hospital, Petach Tikva, Israel, ⁸Pediatric Imaging Unit, Schneider Children's Medical Center of Israel, Petach Tikva, Israel, ⁹Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel
Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are a spectrum of rare autoinflammatory diseases caused by gain-of-function mutations inthe NLRP3 gene. These mutations cause inflammasome hyperactivity and…
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: 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: 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…

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