Abstracts tagged "Autoinflammatory diseases"

Abstract Number: 1062 • ACR Convergence 2021
Telemedicine Enriched Care Model to Optimize Care for Patients with Autoinflammatory Diseases
Lea Oefelein¹, Jens Klotsche², Susanne Benseler³, Jasmin Kuemmerle-Deschner¹ and Tatjana Welzel¹, ¹Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, ²German Rheumatism Research Center, Berlin, Germany, ³University of Calgary, Calgary, AB, Canada
Background/Purpose: Autoinflammatory diseases (AID) are severe potentially life-threatening conditions requiring personalized therapy and monitoring which only few expert centers can provide. Long travel distances impede…
Abstract Number: 1618 • ACR Convergence 2021
Disease Flares in CANDLE/PRAAS with Dose Reductions of Baricitinib
Kader Cetin Gedik¹, Grace Materne², Ana Ortega-Villa³, Gina Montealegre Sanchez⁴, Adam Reinhardt⁵, Paul Brogan⁶, Yackov Berkun⁷, Sara Murias⁸, Maria Robles⁹, Susanne Schalm¹⁰, Adriana Almeida de Jesus¹¹ and Raphaela Goldbach-Mansky¹², ¹Translational Autoinflammatory Diseases Section (TADS)/NIAID/NIH, Bethesda, MD, ²Translational Autoinflammatory Diseases Section, NIAID/NIH, Bethesda, TN, ³Biostatistics Research Branch, Division of Clinical Research, NIAID/NIH, Bethesda, ⁴NIAID/NIH, Bethesda, MD, ⁵Boys Town National Research Hospital, Omaha, NE, ⁶UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁷Hadassah Hebrew University Medical Center, Jerusalem, Israel, ⁸Hospital Infantil La Paz, Madrid, Spain, ⁹Eskenazi Health Center, IndianaPolis, IN, ¹⁰Rheumatologie im Zentrum, Munich, Germany, ¹¹TADS/NIAID/NIH, Silver Spring, MD, ¹²NIH/NIAID, Potomac, MD
Background/Purpose: Patients with chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures /proteasome-associated autoinflammatory syndrome (CANDLE/PRAAS) respond to treatment with baricitinib but require higher exposure…
Abstract Number: 0043 • ACR Convergence 2021
Takinib Inhibits IL-1β-Induced Activation of Signal Transducer and Activator of Transcription 3 (STAT3) in Human Rheumatoid Arthritis Synovial Fibroblasts
Anil singh¹, Paul Panipinto¹, Ruby Siegel¹, Farheen Shaikh¹, Mukesh Chourasia² and Salah-uddin Ahmed¹, ¹Washington State University, Spokane, WA, ²Amity University, Noida, India
Background/Purpose: Interleukin-1 (IL-1) is a crucial mediator of inflammatory cartilage and bone destruction in rheumatoid arthritis (RA). IL-1β signaling relies on the activation of TGF-beta…
Abstract Number: 1083 • ACR Convergence 2021
Outcomes of COVID-19 Illness in Systemic Autoinflammatory Diseases and Changes in Flares During the COVID-19 Pandemic: An International Survey
Samira Nazzar Romero¹, Lakshmi Moorthy², Jennifer Tousseau³, Sivia Lapidus⁴, Mariana Correia Marques⁵, Leanne Mansfield⁶, Marinka Twilt⁷, Grant Schulert⁸, Maria Gutierrez⁹, Saskya Angevare¹⁰, Fatma Dedeoglu¹¹ and Karen Durrant¹², ¹University of California Los Angeles, Los Angeles, CA, ²Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, ³Autoinflamamtory Alliance, Lincoln, CA, ⁴The Joseph M. Sanzari Children's Hospital, Hackensack Meridian Health, Montclair, NJ, ⁵UPMC Children`s Hospital of Pittsburgh, Pittsburgh, PA, ⁶Hospital for Special Surgery, New York, NY, ⁷Alberta Children's Hospital, Calgary, AB, Canada, ⁸Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁹John's Hopkins, Baltimore, MD, ¹⁰Autoinflammatory Alliance / Kaisz / ENCA as patient organizations, Amersfoort, Netherlands, ¹¹Boston Children's Hospital, Boston, MA, ¹²Autoinflammatory Alliance, San Francisco, CA
Background/Purpose: The exaggerated inflammatory responses to the SARS-CoV-2 virus and the paucity of data on COVID-19 infection risk in systemic autoinflammatory disease (SAID) patients posed…
Abstract Number: 1632 • ACR Convergence 2021
Clinical Features and Colchicine Response in Patients with Undifferentiated Systemic Autoinflammatory Disease Carrying E148Q vs. Other MEFV Mutations
Bugra Egeli¹, Holly Wobma¹, Mariana Correia Marques², Jonathan Hausmann³ and Fatma Dedeoglu¹, ¹Boston Children's Hospital, Boston, MA, ²UPMC Children`s Hospital of Pittsburgh, Pittsburgh, PA, ³Beth Israel Deaconess Medical Center, Boston, MA
Background/Purpose: Undifferentiated systemic autoinflammatory diseases (uSAID) are diverse syndromes characterized by acute flares of fever and inflammation, which do not meet clinical criteria for known…
Abstract Number: 0045 • ACR Convergence 2021
Endothelial Cells from Patients with DMARD Naïve, Active Inflammatory Arthritis Demonstrate Pro-inflammatory Sensitisation That Is Reversed by Therapy Initiation
Robert Maughan¹, Marie Lang¹, Anton Olsson¹, Andrew Porter¹, Antonia Greeves², Taryn Youngstein³, Charis Pericleous¹ and Justin Mason¹, ¹Imperial College London, London, United Kingdom, ²Hammersmith Hospital, London, United Kingdom, ³Hammersmith Hospital & Imperial College London, London, United Kingdom
Background/Purpose: Inflammatory arthritides (IA) including rheumatoid arthritis are associated with increased cardiovascular disease (CVD) mortality. Although inflammation-driven endothelial dysfunction is considered a major factor, the…
Abstract Number: 1104 • ACR Convergence 2021
Pathogenic UBA1 Variants in Japanese Patients with Relapsing Polychondritis
Naomi Tsuchida¹, Yosuke Kunishita², Yuri Uchiyama¹, Yohei Kirino³, Kaoru Takase-Minegishi¹, Ryusuke Yoshimi¹, Hideaki Nakajima¹ and Naomichi Matsumoto¹, ¹Yokohama City University Graduate School of Medicine, Yokohama, Japan, ²Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan, ³Yokohama City University, Yokohama, Japan
Background/Purpose: Pathogenic somatic variants in the ubiquitin like modifier activating enzyme 1 gene (UBA1) were discovered in individuals with systemic inflammation of cartilage, skin, and…
Abstract Number: 1707 • ACR Convergence 2021
A Bioengineered Probiotic for the Oral Delivery of an Immunomodulator in Rheumatoid Arthritis
Yuqing Wang¹, Duolong Zhu¹, Laura Ortiz-Velez², Jacob Perry¹, Michael Pennington³, Joseph Hyser¹, Robert Britton¹ and Christine Beeton¹, ¹Baylor College of Medicine, Houston, TX, ²TMC Innovation, Houton, ³AmbioPharm Inc., North Augusta, SC
Background/Purpose: CCR7- effector memory T (TEM) lymphocytes are targets for immunomodulation for the treatment of rheumatoid arthritis (RA). Following activation, TEM cells upregulate the expression…
Abstract Number: 0162 • ACR Convergence 2021
Cardiac Magnetic Resonance Imaging for Guiding Decision-making on Treatment Duration: Data from RHAPSODY, a Phase 3 Clinical Trial of Rilonacept in Recurrent Pericarditis
Paul Cremer¹, David Lin², Alistair Wheeler³, Antonio Abbate⁴, Antonio Brucato⁵, Fang Fang⁶, Antonella Insalaco⁷, Martin LeWinter⁸, Basil S. Lewis⁹, Sushil A. Luis¹⁰, Stephen J. Nicholls¹¹, John Petersen¹², Allan Klein¹, Massimo Imazio¹³ and John F Paolini⁶, ¹Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ²Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ³Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ⁶Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁷Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁸Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁹Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ¹⁰Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²Swedish Medical Center, Seattle, WA, ¹³University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy
Background/Purpose: The magnitude of pericardial delayed hyperenhancement (DHE) by cardiac magnetic resonance imaging (CMR) illustrates the severity of inflammation in pericarditis. We hypothesized that patients…
Abstract Number: 1105 • ACR Convergence 2021
Discovering Variants in Suspected Monogenic Systemic Inflammatory Disease: An Adult Case Series
Jason An¹, Madeline Couse¹, Dilan Dissanayake², Daniela Dominguez², Ronald Laxer¹, Christian Marshall¹, Johannes Roth³, Robert Rottapel⁴ and Linda Hiraki², ¹SickKids, Toronto, ON, Canada, ²The Hospital for Sick Children, Toronto, ON, Canada, ³University of Ottawa, Ottawa, ON, Canada, ⁴St Michael's Hospital, Toronto, ON, Canada
Background/Purpose: Monogenic systemic inflammatory diseases (MSID) are a heterogeneous group of rare conditions caused by single gene variants leading to immune dysregulation. Diagnostic yield of…
Abstract Number: 1769 • ACR Convergence 2021
BMS-986256, an Oral Novel Toll-like Receptor 7 and 8 (TLR7/8) Inhibitor, Does Not Affect the Pharmacokinetics of Mycophenolate Mofetil in Healthy Subjects
Manoj Chiney¹, Ihab Girgis², Melanie Harrison¹, Xiaoping Zhang¹, Yun Shen³, Michelle Dawes¹, Lixian Dong², Diane Shevell³, Urvi Aras¹ and Bindu Murthy³, ¹Bristol Myers Squibb, Lawrenceville, NJ, ²Bristol Myers Squibb, Princeton, NJ, ³Bristol Myers Squibb, Lawrenceville
Background/Purpose: Toll like receptor (TLR) 7 and TLR8 are activated as part of the disease pathophysiology of systemic lupus erythematosus (SLE), including lupus nephritis (LN),…
Abstract Number: 0189 • ACR Convergence 2021
Tapering and Discontinuation of Background Therapies During the Transition to Rilonacept Monotherapy in RHAPSODY, a Phase 3 Clinical Trial of Rilonacept in Patients with Recurrent Pericarditis
Antonio Brucato¹, Alistair Wheeler², Sushil A. Luis³, Antonio Abbate⁴, Paul Cremer⁵, Fang Fang⁶, Antonella Insalaco⁷, Martin LeWinter⁸, Basil S. Lewis⁹, David Lin¹⁰, Stephen J. Nicholls¹¹, Allan Klein⁵, Massimo Imazio¹² and John F Paolini⁶, ¹Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ²Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda, ³Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ⁶Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁷Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁸Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁹Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ¹⁰Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy
Background/Purpose: Post-episode tapering of Standard of Care (SoC) medication in patients with recurrent pericarditis (RP) varies considerably. Gradual tapering of corticosteroids (CS) is recommended in…
Abstract Number: 1106 • ACR Convergence 2021
Phenotypes and Genotypes of NLRP3-AID in Chinese Adult Patients
Na Wu, Di Wu, Junke Miao, Mengzhu Zhao and Min Shen, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China (People's Republic)
Background/Purpose: NLRP3-associated autoinflammatory disease (NLRP3-AID, OMIM 606416), previously called cryopyrin-associated periodic syndrome (CAPS), is a spectrum of autosomal dominant inherited diseases associated with NLRP3 gene…
Abstract Number: 0190 • ACR Convergence 2021
Long-term Safety of Canakinumab in Patients with Autoinflammatory Periodic Fever Syndromes – Interim Analysis of the RELIANCE Registry
Jasmin Kuemmerle-Deschner¹, Jorg Henes², Birgit Kortus-Goetze³, Tilmann Kallinich⁴, Prasad T. Oommen⁵, Juergen Rech⁶, Frank Weller-Heinemann⁷, Gerd Horneff⁸, Ivan Foeldvari⁹, Aleš Janda¹⁰, Catharina Schuetz¹¹, Frank Dressler¹², Michael Borte¹³, Markus Hufnagel¹⁴, Axel Braner¹⁵, Florian Meier¹⁶, Michael Fiene¹⁷, Julia Weber-Arden¹⁸ and Norbert Blank¹⁹, ¹Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, ²University Hospital Tuebingen, Tuebingen, Germany, ³Division of Nephrology, University of Marburg, Marburg, Germany, ⁴Charité University Medicine, Nuremberg, Germany, ⁵Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁶Friedrich-Alexander University (FAU) Erlangen-Nuernberg and Universitaetsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology, Germany, Erlangen, Germany, ⁷Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁸Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁹Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹⁰Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ¹¹Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ¹²Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ¹³ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Leipzig, Germany, ¹⁴Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁵Department of Medicine II, Rheumatology, University Hospital Frankfurt, Germany, Frankfurt a.M., Germany, ¹⁶1 Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany 2 Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt am Main, Germany, Frankfurt a.M., Germany, ¹⁷Department of Internal Medicine, District Hospital Demmin, Demmin, Germany, ¹⁸Novartis Pharma GmbH, Nuremberg, Germany, ¹⁹Rheumatology, University Hospital Heidelberg, Eppelheim, Germany
Background/Purpose: Autoinflammatory periodic fever syndromes (PFS) are characterized by severe systemic and organ inflammation. In clinical trials, successful treatment was achieved with the interleukin-1β inhibitor…
Abstract Number: 1107 • ACR Convergence 2021
Clinical and Genetic Features of Chinese Adult Patients with Chronic Non-bacterial Osteomyelitis: A Single Center Report
Mengzhu Zhao, Min Shen, Di Wu and Keyi Yu, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College, Beijing, China (People's Republic)
Background/Purpose: Chronic non-bacterial osteomyelitis (CNO) is a rare polygenic autoinflammatory bone disease. CNO has been seldom reported in the Chinese population. We aimed to characterize…

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