Abstracts tagged "Innate Immunity Rheumatic Disease"

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: 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…
Abstract Number: 0825 • ACR Convergence 2022
Predicting Outcomes in Macrophage Activation Syndrome (MAS): A Retrospective Study on 43 Consecutive Patients with MAS
Sumanth Madan¹, Suma Balan¹, MITHUN CHALAKKARAYIL BHAGAVALDAS², Spoorthy DR¹, Sudeep Rath¹, Joslyn Thattil¹, Amisha Shahul¹ and Niveditha Kartha¹, ¹Amrita Institute of Medical Sciences, Kochi, India, ²Amrita Institute of Medical Sciences, Muvattupuzha, India
Background/Purpose: Macrophage activation syndrome is a diagnosis along the spectrum of hemophagocytic lymphohistiocytosis (HLH) spectrum disorders. It is a rare, yet potentially fatal disease characterized…
Abstract Number: 1119 • ACR Convergence 2022
Investigating Macrophage Heterogeneity in the Esophagus and Lungs of SSc Patients
Hadijat Makinde¹, Carla Cuda¹, Miranda Gurra¹, Mary Carns², Kathleen Aren², Gaurav Gadhvi¹, Salina Dominguez¹, Jane Dematte³, Darren Brenner⁴, John Pandolfino⁵, G. R. Scott Budinger⁴, Deborah Winter⁶ and Harris Perlman¹, ¹Northwestern University, Chicago, IL, ²Northwestern University Division of Rheumatology, Chicago, IL, ³Northwestern University, Elmhurst, IL, ⁴Northwestern University Feinberg School of Medicine, Chicago, IL, ⁵Northwestern University, Feinberg School of Medicine, Wilmette, IL, ⁶Northwestern University, Skokie, IL
Background/Purpose: Our group has made important contributions to an emerging understanding of monocytes and macrophages as central to SSc pathogenesis. There are numerous studies that…
Abstract Number: 1744 • ACR Convergence 2022
Immune Responses to mRNA Vaccines Against SARS-CoV-2 in Patients with Immune-mediated Inflammatory Rheumatic Diseases
Cristiana Sieiro Santos¹, Sara Calleja Antolín¹, Clara Moriano Morales², Carolina Álvarez Castro¹, Elvira Díez Álvarez¹ and Jose Maria Ruiz de Morales¹, ¹Complejo Asistencial Universitario de León, León, Spain, ²Hospital León, León, Spain
Background/Purpose: Patients with immune-mediated rheumatic diseases (IMRDs) are commonly treated with immunosuppressors and prone to infections. Recently introduced mRNA SARS-CoV-2 vaccines have demonstrated extraordinary efficacy…
Abstract Number: 1842 • ACR Convergence 2022
Vaccination in Patients with Autoinflammatory Periodic Syndromes Under Canakinumab – Safety Data Interim Analysis of the RELIANCE Registry
Jasmin B. Kuemmerle-Deschner¹, Joerg Henes², Birgit Kortus-Goetze³, Tilmann Kallinich⁴, Prasad T. Oommen⁵, Juergen Rech⁶, Tobias Krickau⁷, Frank Weller-Heinemann⁸, Gerd Horneff⁹, Ales Janda¹⁰, Ivan Foeldvari¹¹, Catharina Schuetz¹², Frank Dressler¹³, Michael Borte¹⁴, Markus Hufnagel¹⁵, Florian Meier¹⁶, Michael Fiene¹⁷, Julia Weber-Arden¹⁸ and Norbert Blank¹⁹, ¹Department of Pediatrics, Division of Pediatric Rheumatology, University Hospital Tübingen, Tübingen, Germany, ²Center for Interdisciplinary Clinical Immunology, Rheumatology and Auto-inflammatory Diseases (INDIRA), University Hospital Tübingen, Tübingen, Germany, ³Division of Nephrology, University of Marburg, Marburg, Germany, ⁴Charité - Universitätsmedizin Berlin, Nuremberg, Germany, ⁵Clinic of Pediatric Hematology, Oncology and Clinical Immunology, Heinrich-Heine-University Duesseldorf, Duesseldorf, Germany, ⁶University Clinic Erlangen, Erlangen, Germany, ⁷Friedrich-Alexander Universität Erlangen-Nürnberg (FAU), Pediatrics, Erlangen, Germany, ⁸Klinikum Bremen-Mitte, Prof. Hess Kinderklinik, Bremen, Germany, ⁹Pediatrics, Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ¹⁰Department of Pediatrics, University Hospital Ulm, Ulm, Germany, ¹¹Hamburger Zentrum für Kinder- und Jugendrheumatologie, Hamburg, Germany, ¹²Pediatrics, Medizinische Fakultaet Carl Gustav Carus, Technische Universitaet Dresden, Dresden, Germany, ¹³Division of Pediatric Pneumology, Allergology and Neonatology, Hannover Medical School, Hannover, Germany, ¹⁴ImmunoDeficiencyCenter Leipzig (IDCL), Hospital St. Georg gGmbH Leipzig, Germany, Leipzig, Sachsen, Germany, ¹⁵Division of Pediatric Infectious Diseases and Rheumatology, Department of Pediatrics and Adolescent Medicine, University Hospital Medical Center Freiburg, Medical Faculty, University of Freiburg, Freiburg, Germany, ¹⁶Division 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, ¹⁷Rheumatology Center Greifswald, Greifswald, Germany, ¹⁸Novartis Pharma GmbH, Nuernberg, Germany, ¹⁹Rheumatology, University Hospital Heidelberg, Heidelberg, Germany
Background/Purpose: Treatment of autoinflammatory periodic syndromes with the interleukin-1β inhibitor canakinumab (CAN) has been shown to be safe and effective in clinical trials and in…
Abstract Number: 2213 • ACR Convergence 2022
Multi-omic Analysis of Macrophage Activation Syndrome Associated with sJIA Reveals a Potential Role of Type I Interferons in the Expansion of Cycling T Cells
Kailey Brodeur¹, Liang Chen¹, zhengping huang², Yan Du¹, Holly Wobma³, Maria Taylor⁴, Joyce Chang³, Megan Day-Lewis³, Fatma Dedeoglu³, Olha Halyabar³, Mindy Lo³, Jane W. Newburger⁵, Mary Beth F. Son³, Robert Sundel³, Peter Nigrovic³, lauren henderson³ and Pui Lee³, ¹Boston Children's Hospital, Boston, MA, ²Guangdong Second Provincial Hospital, Guangzhou, China, ³Division of Immunology, Boston Children's Hospital, Boston, MA, ⁴Division of Immunology, Boston Children's Hospital, Brighton, MA, ⁵Department of Cardiology, Boston Children's Hospital, Harvard Medical School, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a complication of systemic juvenile idiopathic arthritis (sJIA) characterized by cytokine storm and overt immune cell activation. We aim…
Abstract Number: 0510 • ACR Convergence 2021
Fibrinogen Modified with Malondialdehyde-Acetaldehyde Adduct (MAA) And/or Citrulline (CIT) Induces Unique Cellular Responses in Human RA Synoviocytes
Brittany Wordekemper, Nozima Aripova, Michael Duryee, Eric Daubach, Bryant England, James O'Dell, Ted Mikuls and Geoffrey Thiele, University of Nebraska Medical Center, Omaha, NE
Background/Purpose: Malondialdehyde (MDA) is produced in response to oxidative stress and is associated with inflammation and disease pathogenesis. MDA can break down and form acetaldehyde…
Abstract Number: 0948 • ACR Convergence 2021
Immunometabolism of Neutrophils in Antiphospholipid Syndrome (APS)
Ajay Tambralli¹, Alyssa Harbaugh², Shanea Estes², Srilakshmi Yalavarthi², Claire Hoy², Gautam Sule² and Jason Knight², ¹University of Michigan, Ypsilanti, MI, ²University of Michigan, Ann Arbor, MI
Background/Purpose: Neutrophil hyperactivity and neutrophil extracellular trap (NET) release (NETosis) appear to play roles in APS pathophysiology. Until recently, it was thought that neutrophils relied…
Abstract Number: 1518 • ACR Convergence 2020
The Role for Neutrophils in the Early Phases of Enthesitis in Spondyloarthritis
Zheni Stavre¹, Charles Bridgewood², Qiao Zhou², Yukiko Maeda³, Jozsef Karman⁴, Dennis McGonagle⁵ and Ellen Gravallese⁶, ¹University of Massachusetts Medical School-Rheumatology Division, Worcester, MA, ²University of Leeds, Leeds, United Kingdom, ³University of Massachusetts Medical School, worcester, MA, ⁴Abbvie Inc., Worcester, MA, ⁵The University of Leeds, Leeds Institute for Rheumatic and Musculoskeletal Medicine, NIHR Leeds Biomedical Research Centre, Leeds Teaching Hospitals Trust, Leeds, UK, Leeds, United Kingdom, ⁶Brigham and Women's Hospital, Boston, MA
Background/Purpose: Neutrophils are present in the early phases of spondyloarthritis (SpA)-associated uveitis, skin and intestinal disease, but their role in enthesitis remains unknown. We investigated…

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