Abstracts tagged "Autoinflammatory diseases"

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…
Abstract Number: 0191 • ACR Convergence 2021
Influence of Canakinumab Dosing on Long-term Efficacy and Safety in Patients with Cryopyrin-associated Periodic Syndromes (CAPS) – 30-months Interim Analysis of the RELIANCE Registry
Jasmin Kuemmerle-Deschner¹, Birgit Kortus-Goetze², Prasad T. Oommen³, Aleš Janda⁴, Juergen Rech⁵, Tilmann Kallinich⁶, Frank Weller-Heinemann⁷, Gerd Horneff⁸, Ivan Foeldvari⁹, Catharina Schuetz¹⁰, Michael Borte¹¹, Axel Braner¹², Julia Weber-Arden¹³ and Norbert Blank¹⁴, ¹Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany, ²Division of Nephrology, University of Marburg, Marburg, Germany, ³Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁴Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ⁵Friedrich-Alexander University (FAU) Erlangen-Nuernberg and Universitaetsklinikum Erlangen, Department of Internal Medicine 3 - Rheumatology and Immunology, Germany, Erlangen, Germany, ⁶Charité University Medicine, Nuremberg, 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, ¹⁰Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ¹¹ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Leipzig, Germany, ¹²Department of Medicine II, Rheumatology, University Hospital Frankfurt, Germany, Frankfurt a.M., Germany, ¹³Novartis Pharma GmbH, Nuremberg, Germany, ¹⁴Rheumatology, University Hospital Heidelberg, Eppelheim, Germany
Background/Purpose: The IL-1 inhibitor canakinumab (CAN) induces rapid remission of symptoms of CAPS (Cryopyrin-associated periodic syndromes), a monogenic autoinflammatory disease with severe systemic inflammation, both…
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: 0192 • ACR Convergence 2021
Influence of Canakinumab Dosing on Efficacy and Safety of Long-term Treatment in Patients with Familial Mediterranean Fever – Interim Analysis of the RELIANCE Registry
Jorg Henes¹, Norbert Blank², Tilmann Kallinich³, Frank Dressler⁴, Gerd Horneff⁵, Ivan Foeldvari⁶, Markus Hufnagel⁷, Birgit Kortus-Goetze⁸, Frank Weller-Heinemann⁹, Florian Meier¹⁰, Julia Weber-Arden¹¹ and Jasmin Kuemmerle-Deschner¹², ¹University Hospital Tuebingen, Tuebingen, Germany, ²Rheumatology, University Hospital Heidelberg, Eppelheim, Germany, ³Charité University Medicine, Nuremberg, Germany, ⁴Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ⁵Paediatric Rheumatology International Trials Organisation (PRINTO), Sankt Augustin, Germany, ⁶Hamburger Zentrum fuer Kinder- und Jugendrheumatologie, Hamburg, Germany, ⁷Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ⁸Division of Nephrology, University of Marburg, Marburg, Germany, ⁹Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ¹⁰1 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, ¹¹Novartis Pharma GmbH, Nuremberg, Germany, ¹²Pediatric Rheumatology and Autoinflammatory Reference Center, University Children's Hospital Tuebingen, Tuebingen, Germany
Background/Purpose: Familial Mediterranean Fever (FMF) is characterized by severe systemic and organ inflammation. Successful treatment with rapid remission of symptoms and normalization of laboratory parameters…
Abstract Number: 1116 • ACR Convergence 2021
Disease Characteristics of Steroid-Induced Spinal Epidural Lipomatosis: A Systematic Review
Haseeb Chaudhary¹, Usama Nasir², Khezar Syed³, Abigayle Sullivan³, Shilla Zachariah³, Christian AkemDimala³, Muhammad Khan⁴ and Anthony Donato³, ¹Tower Health System, Reading, PA, ²Reading Hospital, Tower Health System, Reading, PA, ³Reading Hospital, Tower Health System, Reading, PA, ⁴University of Kentucky, Lexington, KY
Background/Purpose: Steroid-induced epidural lipomatosis (SEL) is a rare phenomenon causing neuronal compression from adipose tissue deposition in the epidural space. We analyzed the available literature…

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