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Abstracts tagged "Innate Immunity Rheumatic Disease"

  • Abstract Number: 0797 • ACR Convergence 2023

    Innate Lymphoid Cells Enhance Development of CD4+ T-cell Driven Autoimmune Arthritis

    Agnieszka Lastowska1, Charlotte E. van der Plas1, Anders Nguyen1, Symeon Kourmoulakis1, Miriam Bollmann1 and Mattias N.D Svensson2, 1Department of Rheumatology and Inflammation research, Sahlgrenska Academy, University of Gothenburg, Gothenburg, Sweden, 2University of Gothenburg, Gothenburg, Sweden

    Background/Purpose: Innate Lymphoid cells (ILCs) are innate counterparts to T-cells that, based on their functional phenotype, can be divided into three subpopulations called Group 1…
  • Abstract Number: 0838 • ACR Convergence 2023

    Infusion of Etanercept into the Peripheral Lymphatics Significantly Reduces Disease Activity in Rheumatoid Arthritis Patients with Inadequate Response to Subcutaneous Injections

    Russell Ross1, Vibeke Strand2, Roel Querubin3, John Goldman4, Richard Leff5, Alexis Melson1, Pei-Ling Roerig6 and Alan Smith7, 1Sorrento Therapeutics, Atlanta, GA, 2Stanford University, Portola Valley, CA, 3Marietta Rheumatology Associates, Marietta, GA, 4Northside Hospital, Atlanta, GA, 5Kezar Life Sciences, Chadds Ford, PA, 64P Therapeutics, Orlando, FL, 74P Therapeutics, Atlanta, GA

    Background/Purpose: The lymphatic system serves as a conduit for transporting immune cells and inflammatory molecules present in arthritic joints to lymph nodes (LN), playing a…
  • Abstract Number: 1712 • ACR Convergence 2023

    Identification and Functional Characterization of Eight CANDLE/PRAAS Causing Proteasome Variants in Five Unrelated Patients

    Adriana Almeida de Jesus1, Jonas J. Papendorf2, Frederic Ebstein3, Sara Alehashemi4, Daniela Pioto5, Anna Kozlova6, Maria Teresa TErreri7, Anna Shcherbina6, Andre Rastegar8, Marta Rodrigues9, Renan Pereira10, Sophia Park11, Bin Lin12, Kat Uss11, Ana Flavia da Silva Pina5, FLAVIO SZTAJNBOK13, Sofia Torreggiani14, Jennifer Stoddard15, Julie Niemela15, Sergio Rosenzweig15, Adriana Fonseca16, Marietta De Guzman17, Nicole Micheloni5, Melissa Fraga5, Sandro Perazzio18, Raphaela Goldbach-Mansky19 and Elke Krueger2, 1NIAID, NIH, Bethesda, MD, 2Institut für Medizinische Biochemie und Molekularbiologie (IMBM), Universitätsmedizin Greifswald, Greifswald, Germany, 3Nantes Université, Nantes, France, 4NIH/NIAID/TADS, Clarksville, MD, 5Universidade Federal de São Paulo (UNIFESP), São Paulo, Brazil, 6Center for Pediatric Hematology, Oncology, Immunology, Moscow, Russia, 7UNIFESP, São Paulo, Brazil, 8NIH, Bethesda, MD, 9Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, Brazil, 10Universidade Federal do Rio Grande do Sul, Porto Alegre, Brazil, 11Translational Autoinflammatory Diseases Section, LCIM, NIAID, NIH, Bethesda, MD, 12Translational Autoinflammatory Diseases Section l LCIM, NIAID, NIH, Bethesda, MD, 13UFRJ/UERJ, São Paulo, Brazil, 14University of Maryland Baltimore, Baltimore, MD, 15Department of Laboratory Medicine, Clinical Center, NIH, Bethesda, MD, 16Instituto de Puericultura e Pediatria Martagao Gesteira / Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, 17Baylor College of Medicine, Houston, TX, 18Universidade de Sao Paulo (Unifesp); Universidade de São Paulo (USP); Fleury Laboratories, São Paulo, Brazil, 19NIH/NIAID, Potomac, MD

    Background/Purpose: Mutations in genes coding for 20S proteasome subunits or proteasome assembly helpers cause chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or…
  • Abstract Number: 1932 • ACR Convergence 2023

    The Association Between Initial Ferritin and Complications from Adults Onset Stills Disease and Intensive Care Unit Admission

    Olivia Yang1, Caroline Bui1, Scott Kubomoto1, Napatkamon Ayutyanont1, Kimberly Vickery1 and Kumar Venkat2, 1Riverside Community Hospital, Riverside, CA, 2Retired, Irvine, CA

    Background/Purpose: Adult Onset Still's Disease (AOSD) is an autoinflammatory disease characterized by quotidian fever, arthralgia, and a salmon colored rash. Due to its rarity, there…
  • Abstract Number: 1936 • ACR Convergence 2023

    Higher Rates of Disease Control During the Coronavirus Pandemic in Pediatric Patients with Autoinflammatory Periodic Diseases on Canakinumab Treatment – Interim Data from the RELIANCE Registry

    Gerd Horneff1, Norbert Blank2, Jasmin B. Kuemmerle-Deschner3, Joerg Henes4, Birgit Kortus-Goetze5, Prasad T. Oommen6, Anne Pankow7, Tobias Krickau8, Catharina Schuetz9, Ivan Foeldvari10, Juergen Rech11, Frank Weller-Heinemann12, Ales Janda13, Markus Hufnagel14, Florian M. Meier15, Frank Dressler16, Michael Borte17, Ioana Andreica18, Peter Wasiliew19, Michael Fiene20, Daniel Windschall21, Martin Krusche22, Tania Kuempfel23, Julia Weber-Arden24 and Tilmann Kallinich25, 1Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, 2University Hospital Heidelberg, Heidelberg, Germany, 3med.uni-tuebingen, Tübingen, Germany, 4University Hospital Tuebingen, Tuebingen, Germany, 5Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, 6Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 7Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, 8Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, 9Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, 10Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 11University Clinic Erlangen, Erlangen, Germany, 12Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, 13Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, 14Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, 15Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, 16Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, 17Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, 18Rheumazentrum Ruhrgebiet Herne, Herne, Germany, 19Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 20Rheumatology Center Greifswald, Greifswald, Germany, 21Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, 22UKE, Hamburg, Germany, 23Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, 24Novartis Innovative Medicines, Nuernberg, Germany, 25Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany

    Background/Purpose: Pediatric patients with autoinflammatory diseases (AID) on Canakinumab (CAN) therapy have been affected by the coronavirus pandemic including SARS-CoV-2 infection, SARS-CoV-2 vaccination, and AID…
  • Abstract Number: 2358 • ACR Convergence 2023

    Neutrophil Extracellular Traps Induce Endothelial-to-Mesenchymal Transition and Associate with Vascular Complications in Scleroderma

    Ramadan Ali1, Wenying Liang1, Claire Shiple1, Rosemary Gedert2, Suiyuan Huang1, Cyrus Sarosh1, Eliza Pei-Suen Tsou1, Dinesh Khanna1 and Jason Knight1, 1University of Michigan, Ann Arbor, MI, 2University of Michigan, Onsted, MI

    Background/Purpose: Systemic sclerosis (SSc, also known as scleroderma) is a disease characterized by fibrosis, autoimmunity, and vasculopathy. Many devastating complications such as digital ulcerations, scleroderma…
  • Abstract Number: 2369 • ACR Convergence 2023

    Investigating Macrophage Heterogeneity in the Esophagus of SSc Patients

    Hadijat Makinde1, Matthew Dapas2, Salina Dominguez2, Tyler Therron1, Miranda Gurra1, Mary Karns3, Kathleen Aren3, Alexandra Soriano2, Lutfiyya Muhammad4, Carla Cuda1, Dustin Carlson1, Jane Dematte5, Darren Brenner1, John Pandolfino6, Harris Perlman1, Deborah Winter7 and Marie-Pier Tetreault1, 1Northwestern University, Chicago, IL, 2Northwestern University FSM Division of Rheumatology, Chicago, IL, 3Northwestern University Division of Rheumatology, Chicago, IL, 4Northwestern University Feinberg School of Medicine, Chicago, IL, 5Northwestern University, Elmhurst, IL, 6Northwestern University, Feinberg School of Medicine, Wilmette, IL, 7Northwestern University, Skokie, IL

    Background/Purpose: Systemic sclerosis (SSc) is characterized by an initial inflammatory phase followed by fibrosis. Esophageal dysfunction in SSc is associated with Gastroesophageal Reflux Disease (GERD),…
  • Abstract Number: 0052 • ACR Convergence 2023

    Possible Involvement of TLR4 in the Pathogenesis of Primary Sjögren’s Syndrome Through Induction of the Expression of BAFF Receptor, BR3 in Peripheral Monocytes

    Keiko Yoshimoto1, Yumi Ikeda1, Katsuya Suzuki2, Hiroyuki Fukui1, Kotaro Matsumoto1, Masaru Takeshita1, Tsutomu Takeuchi3 and Yuko Kaneko4, 1Keio University School of Medicine, Tokyo, Japan, 2Keio University, Shinjuku, Japan, 3Keio University School of Medicine and Saitama Medical University, Tokyo, Japan, 4Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Tokyo, Japan

    Background/Purpose: Toll-like receptors (TLRs) play a pivotal role in innate immune system and chronic inflammation in autoimmune diseases. It has been reported that signaling pathways…
  • Abstract Number: 0061 • ACR Convergence 2023

    Discovery of a RIPK2 Scaffolding Inhibitor for the Treatment of Joint Autoimmune Diseases

    Marta Wlodarska1, Chad Van Huis2, Florian Hoss3, Rosana Meyer1, Xiaokang Lu2, Dominik Koelmel2, Brian Sanchez2, Chuck Lesch2, Alissa Telling2, Martin Minns1, Nneka Mbah2, Isabelle Lacan1, Robert Aversa1, Charles Lesburg1, Carmen Yu2, Stephen Soisson1, Natalie Dales1, Darryl Patrick1, Anthony Opipari2, Shifeng Pan4 and Luigi Franchi2, 1Odyssey Therapeutics, Boston, MA, 2Odyssey Therapeutics, Ann Arbor, MI, 3Odyssey Therapeutics, Frankfurt, Germany, 4Odyssey Therapeutics, San Diego, CA

    Background/Purpose: Receptor interacting protein kinase 2 (RIPK2) is a key signaling node for inflammation caused by peptidoglycan (PGN). In the intestine, RIPK2 integrates signaling originating…
  • Abstract Number: 0275 • ACR Convergence 2023

    No Cumulative Effect of Infection Rates in Children Receiving Long-term Canakinumab Treatment in Autoinflammatory Periodic Fever Syndromes − Data from the RELIANCE Registry

    Jasmin B. Kuemmerle-Deschner1, Joerg Henes2, Birgit Kortus-Goetze3, Prasad T. Oommen4, Anne Pankow5, Tilmann Kallinich6, Tobias Krickau7, Catharina Schuetz8, Gerd Horneff9, Ivan Foeldvari10, Juergen Rech11, Frank Weller-Heinemann12, Ales Janda13, Markus Hufnagel14, Florian M. Meier15, Frank Dressler16, Michael Borte17, Ioana Andreica18, Peter Wasiliew19, Michael Fiene20, Daniel Windschall21, Martin Krusche22, Tania Kuempfel23, Julia Weber-Arden24 and Norbert Blank25, 1med.uni-tuebingen, Tübingen, Germany, 2University Hospital Tuebingen, Tuebingen, Germany, 3Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, 4Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 5Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, 6Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany, 7Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, 8Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, 9Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, 10Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 11University Clinic Erlangen, Erlangen, Germany, 12Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, 13Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, 14Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, 15Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, 16Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, 17Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, 18Rheumazentrum Ruhrgebiet Herne, Herne, Germany, 19Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 20Rheumatology Center Greifswald, Greifswald, Germany, 21Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, 22UKE, Hamburg, Germany, 23Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, 24Novartis Innovative Medicines, Nuernberg, Germany, 25University Hospital Heidelberg, Heidelberg, Germany

    Background/Purpose: Autoinflammatory diseases (AID) have been treated safely and effectively with the interleukin-1β inhibitor canakinumab (CAN) in controlled trials and routine clinical practice. The most…
  • Abstract Number: 0276 • ACR Convergence 2023

    Disease Control in Patients with Monogenetic Autoinflammatory Diseases Under Canakinumab Treatment – Comparison of 30 Months Interim Data from the RELIANCE Registry

    Ivan Foeldvari1, Tilmann Kallinich2, Norbert Blank3, Joerg Henes4, Birgit Kortus-Goetze5, Prasad T. Oommen6, Anne Pankow7, Tobias Krickau8, Catharina Schuetz9, Gerd Horneff10, Juergen Rech11, Frank Weller-Heinemann12, Ales Janda13, Markus Hufnagel14, Florian M. Meier15, Frank Dressler16, Michael Borte17, Ioana Andreica18, Peter Wasiliew19, Michael Fiene20, Daniel Windschall21, Martin Krusche22, Tania Kuempfel23, Julia Weber-Arden24 and Jasmin B. Kuemmerle-Deschner25, 1Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 2Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité Universitätsmedizin Berlin, Nuremberg, Germany, 3University Hospital Heidelberg, Heidelberg, Germany, 4University Hospital Tuebingen, Tuebingen, Germany, 5Department of Internal Medicine, Division of Nephrology,University Hospital of Giessen and Marburg, Marburg, Germany, 6Department of Pediatric Oncology, Hematology and Clinical Immunology, Center for Child and Adolescent Health,Medical Faculty Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 7Department of Rheumatology and Clinical Immunology,Charité-Universitätsmedizin Berlin, Berlin, Germany, 8Pediatrics, Friedrich-Alexander University Erlangen-Nuernberg (FAU), Erlangen, Germany, 9Department of Pediatrics, Medizinische Fakultät Carl Gustav Carus,Technische Universität Dresden, Dresden, Germany, 10Asklepios Klinik Sankt Augustin GmbH, Bonn, Germany, 11University Clinic Erlangen, Erlangen, Germany, 12Division of Pediatric Rheumatology, Prof. Hess Children's Hospital, Bremen, Bremen, Germany, 13Department of Pediatrics and Adolescent Medicine, University Medical Center Ulm, Ulm, Germany, 14Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, 15Department of General Pharmacology and Toxicology, Goethe University Hospital and Goethe University Frankfurt, Frankfurt am Main, Germany, 16Department of Paediatric Pneumology, Allergology and Neonatology, Children's Hospital, Hannover Medical School, Hannover, Germany, 17Hospital for Children & Adolescents, St. Georg Hospital, Leipzig, Germany, 18Rheumazentrum Ruhrgebiet Herne, Herne, Germany, 19Division of Pediatric Rheumatology and autoinflammation reference center Tuebingen, Department of Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, 20Rheumatology Center Greifswald, Greifswald, Germany, 21Clinic of Paediatric and Adolescent Rheumatology, St. Josef-Stift Sendenhorst, Northwest German Center for Rheumatology, Sendenhorst, Germany, 22UKE, Hamburg, Germany, 23Institute of Clinical Neuroimmunology, Biomedical Center and University Hospital, Ludwig-Maximilians Universität München, Muenchen, Germany, 24Novartis Innovative Medicines, Nuernberg, Germany, 25med.uni-tuebingen, Tübingen, Germany

    Background/Purpose: Treatment of autoinflammatory periodic diseases (AID) with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in controlled trials and…
  • 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: 0138 • ACR Convergence 2022

    Patients with Familial Mediterranean Fever (FMF) Under Canakinumab Treatment – Long-term Efficacy and Safety Interim Data of the RELIANCE Registry

    Joerg Henes1, Jasmin B. Kuemmerle-Deschner2, Tobias Krickau3, Tilmann Kallinich4, Frank Dressler5, Gerd Horneff6, Florian Meier7, Ivan Foeldvari8, Frank Weller-Heinemann9, Birgit Kortus-Goetze10, Markus Hufnagel11, Juergen Rech12, Prasad T. Oommen13, Julia Weber-Arden14 and Norbert Blank15, 1Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, 2Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, 3Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, 4Charité - Universitätsmedizin Berlin, Nuremberg, Germany, 5Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, 6Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, 7Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany, and Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt, Germany, 8Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 9Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, 10Division of Nephrology, University of Marburg, Marburg, Germany, 11Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, 12University Clinic Erlangen, Erlangen, Germany, 13Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 14Novartis Pharma GmbH, Nuernberg, Germany, 15Rheumatology, University Hospital Heidelberg, Heidelberg, Germany

    Background/Purpose: Familial Mediterranean fever (FMF) is a chronic disease characterized by recurrent episodes of fever and serositis, with a risk of severe complications (e. g.…
  • Abstract Number: 0812 • ACR Convergence 2022

    3-years Safety and Efficacy Outcomes of Canakinumab Treatment in Cryopyrin-associated Periodic Syndromes (CAPS) – Data from the RELIANCE Registry

    Jasmin B. Kuemmerle-Deschner1, Birgit Kortus-Goetze2, Prasad T. Oommen3, Ales Janda4, Juergen Rech5, Catharina Schuetz6, Tilmann Kallinich7, Frank Weller-Heinemann8, Gerd Horneff9, Ivan Foeldvari10, Florian Meier11, Michael Borte12, Tobias Krickau13, Julia Weber-Arden14 and Norbert Blank15, 1Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, 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, 5University Clinic Erlangen, Erlangen, Germany, 6Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, 7Charité - Universitätsmedizin Berlin, Nuremberg, Germany, 8Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, 9Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, 10Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, 11Division of Rheumatology, University Hospital Frankfurt, Goethe University, Frankfurt am Main, Germany, and Fraunhofer Institute for Molecular Biology and Applied Ecology IME, Project Group Translational Medicine and Pharmacology TMP, Frankfurt, Germany, 12ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, Germany, 13Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, 14Novartis Pharma GmbH, Nuernberg, Germany, 15Rheumatology, University Hospital Heidelberg, Heidelberg, Germany

    Background/Purpose: Cryopyrin-associated periodic syndromes (CAPS) are monogenic autoinflammatory diseases with severe systemic inflammation. The IL-1β inhibitor canakinumab (CAN) leads to a rapid remission of CAPS…
  • Abstract Number: 0813 • ACR Convergence 2022

    Canakinumab in Patients with Tumor Necrosis Factor Receptor-associated Periodic Syndrome (TRAPS) – Long-term Efficacy and Safety Data from a RELIANCE Registry Interim Analysis

    Norbert Blank1, Catharina Schuetz2, Joerg Henes3, Tilmann Kallinich4, Prasad T. Oommen5, Michael Borte6, Markus Hufnagel7, Ales Janda8, Julia Weber-Arden9 and Jasmin B. Kuemmerle-Deschner10, 1Rheumatology, University Hospital Heidelberg, Heidelberg, Germany, 2Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, 3Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, 4Charité - Universitätsmedizin Berlin, Nuremberg, Germany, 5Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, 6ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, 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, 8Department of Pediatrics, University Hospital Ulm, Ulm, Germany, 9Novartis Pharma GmbH, Nuernberg, Germany, 10Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany

    Background/Purpose: TRAPS is a rare hereditary autoinflammatory disease characterized by periodic fever and severe systemic and organ inflammation. Successful treatment was achieved with the interleukin-1β…
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