Abstracts tagged "Autoinflammatory diseases"

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…
Abstract Number: 0193 • ACR Convergence 2021
Tocilizumab for the Treatment of Familial Mediterranean Fever – a Randomized, Double Blind, Placebo-controlled Phase II Study
Jorg Henes¹, Sebastian Saur², David Kofler³, Martin Krusche⁴, Theodoros Xenitidis⁵, Christoph Meisner⁶, Claudia Kedor⁷, Ina Koetter⁸, Hendrik Schulze-Koops⁹ and Eugen Feist¹⁰, ¹University Hospital Tuebingen, Tuebingen, Germany, ²Universtiy Hostpiatl Tübingen, Tuebingen, Germany, ³Division of Rheumatology and Clinical Immunology, Department I of Internal Medicine; University Hospital Cologne, Cologne, Germany, ⁴Charite Berlin, Berlin, Germany, ⁵University Hospital Tuebingen, Rheumatology, Tuebingen, Germany, ⁶University Tuebingen, Statistics, Tuebingen, Germany, ⁷Charite University Hospital Berlin, Berlin, Germany, ⁸University Hospital Hamburg Eppendorf, Hamburg, Germany, ⁹Division of Rheumatology and Clinical Immunology, Department of Internal Medicine IV, University of Munich, Munich, Germany, ¹⁰Helios Department of Rheumatology, Vogelsang-Gommern, Germany
Background/Purpose: Familial Mediterranean Fever (FMF) is the most common auto-inflammatory disease characterized by chronic inflammation, recurrent episodes of fever, abdominal and thoracic pain due to…
Abstract Number: 1157 • ACR Convergence 2021
Patient Perspective on the Non Medical Switch of Originator to Its Biosimilar in Inflammatory Arthritis Using a Social Media Survey
David Vega Morales¹, Alejandro Garza-Alpirez² and Carlos Andrés Díaz-Garza², ¹Hospital Universitario, Garcia, Mexico, ²Tecnologico de Monterrey, Escuela de Medicina y Ciencias de la Salud, San Pedro, Mexico
Background/Purpose: The use of biosimilars instead of its originator is a controversial subject with many implications. It is considered that a non medical switch should…
Abstract Number: 0488 • ACR Convergence 2021
Efficacy and Safety of Brepocitinib (Tyrosine Kinase 2/Janus Kinase 1 Inhibitor) for the Treatment of Active Psoriatic Arthritis: Results from a Phase 2b Randomized Controlled Trial
Philip Mease¹, Philip S Helliwell², Paula Silwinska-Stanczyk³, Malgorzata Miakisz⁴, Andrew Ostor⁵, Elena Peeva⁶, Michael Vincent⁶, Vanja Sikirica⁷, Randall Winnette⁸, Ruolun Qiu⁶, Gang Li⁷, Gang Feng⁶, Jean Beebe⁶ and David Martin⁶, ¹Swedish Medical Center/Providence St. Joseph Health and University of Washington, Seattle, WA, ²Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, ³REUMATIKA – Centrum Reumatologii NZOZ, Warsaw, Poland, ⁴Twoja Przychodnia Centrum Medyczne Nowa Sol, Nowa Sol, Poland, ⁵Monash University, Cabrini Hospital & Emeritus Research, Melbourne, Australia, ⁶Pfizer Inc, Cambridge, MA, ⁷Pfizer Inc, Collegeville, PA, ⁸Pfizer Inc, New York, NY
Background/Purpose: Brepocitinib is a small-molecule tyrosine kinase 2/Janus kinase 1 inhibitor that has shown promising results in an oral formulation for plaque psoriasis and alopecia…
Abstract Number: 1432 • ACR Convergence 2021
Modulation of Auto-Inflammation with a Novel Selective Cyclic GMP-AMP Synthase (cGAS) Inhibitor in a Trex1-/- Model
Kelly Pike¹, Alexandre Caron¹, Emilie Bérubé¹, Ramsay Beveridge¹, Marc-Oliver Boily¹, Jason Burch¹, Valerie Dumais¹, Nadine Fradet¹, Samuel Gaudreault¹, Daniel McKay¹, Marianne Raymond¹, Eleftheria Seliniotakis¹, Daniel Sietsema³, Alexander Skeldon¹, Miguel St.-Onge¹, Li Wang² and Michael Crackower², ¹Ventus Therapeutics, Montréal, QC, Canada, ²Ventus Therapeutics, Waltham, MA

Background/Purpose: The detection of viral nucleic acids (NA) elicits a transient type I interferon (IFN) response central to antiviral immunity. Chronic type I IFN responses…
Abstract Number: 0537 • ACR Convergence 2021
The Clinical, Oral Small Molecule IRAK4 Inhibitor, GS-5718, Improves Survival and Reduces Disease Pathologies by Modulating Multiple Inflammatory Endpoints in the Murine NZB/W Model of Spontaneous Lupus
Adam Yadon¹, Vanessa Gorney², Angie Hammond³, Ethan Grant² and Astrid Clarke³, ¹Gilead Sciences, Inc., Seattle, WA, ²Gilead Sciences, Inc, Foster City, CA, ³Gilead Sciences, Inc, Seattle, WA
Background/Purpose: Lupus is a heterogenous autoimmune disease characterized by loss of immune tolerance, production of nucleic acid:autoantibody immune complexes, immune cell hyperactivation, and increased proinflammatory…
Abstract Number: 1452 • ACR Convergence 2021
COVID-19 Infection Among Autoimmune/Auto-inflammatory Rheumatic Disease Patients: Data from an Observational Study
Clio Mavragani¹, Athanasios-Dimitrios Bakasis², Kyriaki Boki³, Athanasios Tzioufas⁴, Panayiotis Vlachoyiannopoulos¹, Ioanna Stergiou⁵, Fotini Skopouli⁶ and Haralampos Moutsopoulos⁷, ¹National and Kapodistrian University of Athens, Athens, Greece, ²1 Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, Athens, Greece, ³3 Rheumatology Unit, Sismanoglio General Hospital, Athens, Greece, Athens, Greece, ⁴Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece, ⁵Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, Athens, ⁶Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, Athens, Greece, ⁷Athens Academy of Athens, Chair Medical Sciences/Immunology, Athens, Greece
Background/Purpose: The impact of COVID-19 infection in patients with autoimmune/auto-inflammatory rheumatic diseases (AARD) under immunomodulatory treatment is not entirely clear and deeper knowledge is of…
Abstract Number: 0539 • ACR Convergence 2021
Vgll3-transgenic Autoimmune Mice Display Features of Cutaneous Fibrosis
Mehrnaz Gharaee-Kermani¹, Allison Billi², Marisa Hildebrandt², Jacob Martens³, Rachael Wasikowski², Joanne (Michelle) Kahlenberg¹ and Johann Gudjonsson², ¹Internal Medicine - Division of Rheumatology and Department of Dermatology, University of Michigan, Ann Arbor, MI, ²Department of Dermatology, University of Michigan, Ann Arbor, MI, ³Graduate Program in Immunology, University of Michigan, Ann Arbor, MI, USA, Ann Arbor, MI
Background/Purpose: Fibrosis is characterized by collagen deposition, fibro/myofibroblast accumulation, and extracellular matrix remodeling and can lead to disfiguring skin changes. In cutaneous lupus, scar formation…
Abstract Number: 1454 • ACR Convergence 2021
RHAPSODY: Rilonacept, an IL-1α and IL-1β Trap, Resolves Pericarditis Episodes and Reduces Risk of Recurrence in a Phase 3 Trial of Patients with Recurrent Pericarditis
Allan Klein¹, Massimo Imazio², Paul Cremer¹, Antonio Brucato³, Antonio Abbate⁴, Fang Fang⁵, Antonella Insalaco⁶, Martin LeWinter⁷, Basil S. Lewis⁸, David Lin⁹, Sushil A. Luis¹⁰, Stephen J. Nicholls¹¹, Arian Pano⁵, Alistair Wheeler¹², Liangxing Zou⁵ and John F Paolini⁵, ¹Department of Cardiovascular Imaging, Center for the Diagnosis and Treatment of Pericardial Diseases, Heart and Vascular Institute, Cleveland Clinic, Cleveland, OH, ²University Cardiology, Cardiovascular and Thoracic Department, AOU Città della Salute e della Scienza di Torino, Torino, Italy, ³Department of Biomedical and Clinical Science, University of Milano, Fatebenefratelli Hospital, Milan, Italy, ⁴VCU Pauley Heart Center, Virginia Commonwealth University, Richmond, VA, ⁵Kiniksa Pharmaceuticals Corp., Lexington, MA, ⁶Department of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children's Hospital, Rome, Italy, ⁷Cardiology Unit, The University of Vermont Medical Center, The University of Vermont, Burlington, VT, ⁸Cardiovascular Clinical Research Institute, Lady Davis Carmel Medical Center, Haifa, Israel, ⁹Minneapolis Heart Institute at Abbott Northwestern Hospital, Minneapolis, MN, ¹⁰Division of Cardiovascular Ultrasound, Department of Cardiovascular Medicine, Mayo Clinic, Rochester, NY, ¹¹MonashHeart, Department of Cardiology, Monash University, Clayton, Australia, ¹²Kiniksa Pharmaceuticals, Ltd., Hamilton, Bermuda
Background/Purpose: Recurrent pericarditis (RP) is an autoinflammatory disease with no FDA-approved therapies. RHAPSODY, a global Phase 3 study, evaluated rilonacept, a once-weekly IL-1α/IL-1β trap, in…

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