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Abstracts tagged "Still’s disease"

  • Abstract Number: 0098 • ACR Convergence 2020

    CCL2 and CCR2 in Adult Onset Still’s Disease

    Ju-Yang Jung1, Ji-Won Kim1, Chang-Hee Suh1 and Hyoun-Ah Kim1, 1Ajou university school of medicine, Suwon, Republic of Korea

    Background/Purpose: Adult onset Still's disease (AOSD) is a rare systemic inflammatory disease characterized by a high spiking fever, evanescent rash, arthralgia, generalized lymphadenopathy, and leukocytosis.…
  • Abstract Number: 0174 • ACR Convergence 2020

    Dense Genotyping of Immunologic Loci Identifies CXCR4 as a Novel Susceptibility Locus for Systemic Juvenile Idiopathic Arthritis

    Emily Shuldiner1, Elaine Remmers2, Miranda Marion3, Marc Sudman4, Colleen Satorius5, Patricia Woo6, Sampath Prahalad7, Carl Langefeld8, Susan Thompson9, Wendy Thomson10 and Michael Ombrello11, 1NIAMS, NIH, Bethesda, 2National Human Genome Research Institute (NHGRI), NIH, Bethesda, MD, 3Wake Forest School of Medicine, Winston-Salem, 4Cincinnati Children's Hospital Medical Center, Cincinnati, 5NHGRI, NIH, Bethesda, 6Great Ormond Street Hospital, London, United Kingdom, 7Emory + Children's Pediatric Institute, Atlanta, GA, 8Wake Forest School of Medicine, Winston Salem, NC, 9Cincinnati Children's Hospital Medical Center/Univ of Cincinnati College of Medicine, Cincinnati, OH, 10Centre for Genetics and Genomics Versus Arthritis, Manchester Academic Health Science Centre, The University of Manchester, Manchester, United Kingdom, 11Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a severe, potentially lethal inflammatory condition. It accounts for a disproportionate share of morbidity and mortality among childhood…
  • Abstract Number: 1148 • ACR Convergence 2020

    The SHARE Recommendations on Diagnosis and Treatment of Systemic JIA

    Arjen Leek1, Jordi Anton2, Tadej Avcin3, Fabrizio De Benedetti4, Victor Boom1, Claudia Bracaglia5, Paul Brogan6, Tamas Constantin7, Alessandro Consolaro8, Pavla Dolezalova9, Despina Eleftheriou10, Helen Foster11, Claas Hinze12, Isabelle Koné-Paut13, Kirsten Minden14, Francesca Minoia15, Pierre Quartier16, Angelo Ravelli8, Nicolino Ruperto17, Joost Swart18, Yosef Uziel19, Helmut Wittkowski20, Carine Wouters21, Mojca Zajc Avramovitz22, Nico Wulffraat18 and Sebastiaan Vastert1, 1University Medical Center Utrecht, Utrecht, Netherlands, 2Sant Joan de Déu Hospital, Madrid, Spain, 3Ljubljana University Medical Centre, Ljubljana, Slovenia, 4Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 5Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 6UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 7Semmelweiss University Hospital, Budapest, Hungary, 8Università degli Studi di Genova, Genoa, Italy, 9University Hospital Prague, Prague, Czech Republic, 10UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Section Head Infection, Immunology, and Rheumatology, London, United Kingdom, 11Newcastle University, Newcastle upon Tyne, United Kingdom, 12University Hospital M�nster, M�nster, Germany, 13Necker Hospital, Paris, France, 14Deutsches Rheuma-Forschungszentrum Berlin, Berlin, Germany, 15Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, 16Necker-Enfants Malades University Hospital, Assistance Publique-Hopitaux de Paris, Paris, France, 17Istituto Giannina Gaslini, Genova, Italy, 18IRCCS Istituto Giannina Gaslini, PRINTO, Genoa, Italy, Genova, Italy, 19Meir Medical Center, Kfar Saba, Israel, 20University Hospital Muenster, Muenster, Germany, 21University Hospital Leuven, Leuven, Belgium, 22University Hospital Ljubljana, Ljubljana, Slovenia

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare, complex auto-inflammatory disease with significant morbidity including fever, rash, serositis and articular problems. With the availability…
  • Abstract Number: 1633 • ACR Convergence 2020

    A Randomized, Double-Blind, Placebo-Controlled Study of Anakinra in Pediatric and Adult Patients with Still’s Disease

    Laura Schanberg1, Peter Nigrovic2, Ashley Cooper3, Winn Chatham4, Shoghik Akoghlanian5, Namrata Singh6, C. Egla Rabinovich7, Akaluck Thatayatikom8, Alysha Taxter9, Jonathan Hausmann10, Milan Zdravkovic11, Sven Ohlman11, Henrik Andersson11, Susanna Cederholm11, Gunilla Huledal11, Rayfel Schneider12 and Fabrizio De Benedetti13, 1Duke University Medical Center, Durham, NC, 2Division of Rheumatology, Inflammation and Immunity, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, USA; Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA, Boston, 3Children's Mercy Kansas City, Kansas City, MO, 4University of Alabama at Birmingham, Birmingham, AL, 5Nationwide Children’s Hospital, Columbus, 6University of Washington, Bellevue, WA, 7Duke University Hospital, Durham, NC, 8Department of Pediatrics, University of Florida, Gainesville, FL, 9Wake Forest Baptist Medical Center, Winston-Salem, 10Boston Children's Hospital / Beth Israel Deaconess Medical Center, Cambridge, MA, 11Sobi, Stockholm, Sweden, 12University of Toronto, The Hospital for Sick Children, Toronto, Canada, 13Division of Rheumatology, Laboratory of Immuno-Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy

    Background/Purpose: Still’s disease, including both systemic juvenile idiopathic arthritis (SJIA) and adult-onset Still’s disease (AOSD), is a rare systemic auto-inflammatory disorder associated with an activated…
  • Abstract Number: 1950 • ACR Convergence 2020

    Elevated Serum Gasdermin D N-terminal Implicates Macrophage Pyroptosis in Adult-onset Still’s Disease and Systemic Juvenile Idiopathic Arthritis

    Hideto Nagai1, Yohei Kirino2, Hiroto Nakano3, Yosuke Kunishita1 and Michael Ombrello4, 1Yokohama City University Graduate School of Medicine, Yokohama, Kanagawa, Japan, 2Yokohama City University Graduate School of Medicine, Yokohama, Japan, 3NIAMS, NIH, Bethesda, 4Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD

    Background/Purpose: Elevation of serum IL-18 in adult-onset Still's disease (AOSD) and systemic juvenile idiopathic arthritis (sJIA) suggests involvement of one or more inflammasome in these…
  • Abstract Number: 028 • 2020 Pediatric Rheumatology Symposium

    A Randomized, Double-blind, Placebo-controlled Study of Anakinra in Pediatric and Adult Patients with Still’s Disease

    Laura Schanberg1, Peter Nigrovic 2, Ashley Cooper 3, Winn Chatham 4, Shoghik Akoghlanian 5, Namrata Singh 6, C. Egla Rabinovich 7, Akaluck Thatayatikom 8, Alysha Taxter 9, Jonathan Hausmann 10, Milan Zdravkovic 11, Sven Ohlman 11, Henrik Andersson 11, Susanna Cederholm 12, Gunilla Huledal 11, Rayfel Schneider 13 and Fabrizio De Benedetti 14, 1Duke Children's Hospital & Health Center, Durham, North Carolina, 2Boston Children's Hospital, Boston, Massachusetts, 3Children's Mercy Kansas City, Kansas City, 4University of Alabama at Birmingham, Birmingham, 5Nationwide Children's Hospital, Columbus, 6University of Iowa Hospitals and Clinics, Iowa, Iowa, 7Duke University Hospital, Durham, 8University of Florida, Gainesville, 9Wake Forest Baptist Medical Center, Winston-Salem, 10Division of Immunology, Boston Children's Hospital; Rheumatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, 11Sobi, Stockholm, Sweden, 12Stockholm, Sweden, 13The Hospital for Sick Children, Toronto, Canada, 14Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy

    Background/Purpose: Adult-onset Still’s disease (AOSD) and systemic juvenile idiopathic arthritis (SJIA) are rare systemic disorders of auto-inflammatory nature. There is a growing understanding that SJIA…
  • Abstract Number: 092 • 2020 Pediatric Rheumatology Symposium

    The Initial Treatment of Systemic Juvenile Idiopathic Arthritis: An International Collaboration Among 10 Registries

    Mary Beth Son1, Yukiko Kimura 2, Kristiina Aalto 3, Lillemor Berntson 4, Johnathan Dallas 1, Ciaran Duffy 5, Mia Glerup 6, Jaime Guzman 7, Troels Herlin 8, Petteri Hovi 9, Kimme Hyrich 10, Jens Klotsche 11, Bo Magnusson 12, Vanessa McIntyre 13, Ellen Nordal 14, Seza Ozen 15, Maria Jose Santos 16, Betul Sozeri 17 and Timothy Beukelman 18, 1Boston Children's Hospital, Boston, Massachusetts, 2Hackensack Meridian School of Medicine, Hackensack, 3Department of Pediatrics, New Children's Hospital, Helsinki University Hospital, Pediatric Research Center, University of Helsinki, Helsinki, Finland., HUS, Finland, 4Department of Womens and Childrens Health, Uppsala University, Uppsala, Sweden., Uppsala, Sweden, 5Ottawa, Canada, 6Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark, Aarhus, Denmark, 7University of British Columbia and BC Children's Hospital, Vancouver, Canada, 8Department of Pediatrics, Aarhus University Hospital, Aarhus, Denmark., Aarhus N, Denmark, 9Helsinki University Hospital, Helsinki, Finland, 10Manchester, United Kingdom, 11Berlin, Germany, 12Karolinska University Hospital, Stockholm, Sweden, 13Manchester University, Manchester UK, United Kingdom, 14Department of Pediatrics, University Hospital of North Norway, and Department of Clinical Medicine, UiT The Arctic University of Norway, Tromsø, Norway., Tromsø, Norway, 15Hacettepe University, Division of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, 16Portugal, 17Boston, Turkey, 18University of Alabama at Birmingham, Birmingham

    Background/Purpose: The introduction of biologic medications has revolutionized the care of children with systemic juvenile idiopathic arthritis (SJIA). Differences in treatment approaches among different countries…
  • Abstract Number: L06 • 2019 ACR/ARP Annual Meeting

    Interferon-gamma (IFN-γ) Neutralization with Emapalumab and Time to Response in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (s-JIA) who failed High-Dose Glucocorticoids

    Fabrizio De Benedetti1, Paul Brogan 2, Alexei A. Grom 3, Pierre Quartier 4, Rayfel Schneider 5, Jordi Antón 6, Claudia Bracaglia 7, Manuela Pardeo 8, Giulia Marucci 8, Emanuela Sacco 9, Despina Eleftheriou 10, Charalampia Papadopoulou 11, Philippe Jacqmin 12, maria ballabio 13 and Cristina de Min 13, 1Bambino Gesù Children's Hospital, Rome, Italy, 2Great Ormond Street Hospital for Children NHS Foundation Trust, London, United Kingdom, 3Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 4Necker Hospital, Paris, France, 5Hospital for Sick Children, Toronto, ON, Canada, 6Hospital Sant Joan de Déu, Barcelona, Spain, 7Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, 8Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, 9Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, 10UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section,, london, United Kingdom, 11UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, Infection, Immunology, and Rheumatology section, london, United Kingdom, 12MnS, Dinant, Belgium, 13Sobi AG, basel, Switzerland

    Background/Purpose: MAS is a severe complication of rheumatic diseases, most frequently sJIA and adult-onset Still’s disease. It is characterized by fever, hepatosplenomegaly, liver dysfunction, cytopenias,…
  • Abstract Number: 1247 • 2019 ACR/ARP Annual Meeting

    Adult-Onset Still’s Disease and Spondyloarthritis: Overlapping Syndrome or Incidental Association? A Series of 5 Cases

    Aly Kamissoko 1, Nolan Hassold 2, Alexis Mathian 3, Edouard Pertuiset 4, Gaetane Nocturne 2, Bruno Fautrel 5 and Stéphane Mitrovic6, 1AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Paris, France, Paris, France, 2Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique – Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM, Paris, France., Paris, France, 3AP-HP, Service de Médecine Interne 2, Hôpital Universitaire Pitié-Salpêtrière, Paris, France, Paris, France, 4Rheumatology Department, Pontoise Hospital, Pontoise, France, Pontoise, France, 5Pitié-Salpêtrière Hospital, Department of Rheumatology, AP-HP, Sorbonne University, UPMC university, Paris, Ile-de-France, France, 6AP-HP, Department of Rheumatology, Pitié-Salpêtrière University Hospital, Sorbonne Université, GRC 08, Pierre Louis Institute of Epidemiology and Public Health, Paris, France - Centre d’Etude et de Référence sur les Maladies AutoInflammatoires et les Amyloses (CEREMAIA), FAI2R network, Paris, France - Institut Mutualiste Montsouris, Paris, France, Paris, France

    Background/Purpose: Some forms of spondyloarthritis (SpA) such as SAPHO syndrome are classified as auto-inflammatory disorders. Association with adult onset Still’s disease (AoSD) has never been…
  • Abstract Number: 1324 • 2018 ACR/ARHP Annual Meeting

    Anakinra Treatment in Refractory Cases of Adult-Onset Still Disease: Case Series

    Serdal Ugurlu1, Berna Yurttas1, Gul Guzelant1, Bilgesu Ergezen2 and Huri Ozdogan3, 1Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 2Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey, 3Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Background/Purpose: Despite methotrexate and steroid treatment, in cases of Adult-onset Still’s disease (AOSD) it is usually difficult to maintain clinic stability. In refractory cases, Anakinra…
  • Abstract Number: 1325 • 2018 ACR/ARHP Annual Meeting

    Canakinumab Treatment in Adult-Onset Still’s Disease: Case Series

    Serdal Ugurlu1, Gul Guzelant1, Berna Yurttas1, Bilgesu Ergezen1, Ediz Dalkiliç2, Timucin Kasifoglu3, Burcu Yagız4 and Huri Ozdogan1, 1Istanbul University, Cerrahpasa Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, 2Department of Internal Medicine, Division of Rheumatology, Uludağ University Faculty of Medicine, Bursa, Turkey, 3Department of Internal Medicine, Division of Rheumatology, Eskisehir Osmangazi University Faculty of Medicine, Eskişehir, Turkey, 4Uludag University, Medical Faculty, Department of Internal Medicine, Division of Rheumatology, Bursa, Turkey

    Background/Purpose: In Adult-onset Still’s disease (AOSD), cases refractory to typical DMARDs, Canakinumab (an anti-IL-1ß monoclonal antibody) has been reported to be effective in a limited…
  • Abstract Number: 1414 • 2018 ACR/ARHP Annual Meeting

    Pharmacovigilance of Biologics for Systemic Juvenile Idiopathic Arthritis Patients By the German Biologics Registry

    Gerd Horneff1, Gerd Ganser2, Toni Hospach3, Ivan Foeldvari4, Michael Borte5, Frank Weller-Heinemann6, Kirsten Minden7 and Ariane Klein8, 1Asklepios Kinderklinik St. Augustin GmbH, Sankt Augustin, Germany, 2Klinik für Kinder-und Jugendrheumatologie, Nordwestdeutsches Rheumazentrum, Sendenhorst, Germany, 3Pediatrics, Olgahospital, Klinikum Stuttgart, Stuttgart, Germany, 4Hamburg Centre for Pediatric Rheumatology, Hamburg, Germany, 5St Georg Hospital, Leipzig, Germany, 6PRINTO, Genoa, Italy, 7Charité–Universitätsmedizin Berlin, Berlin, Germany, 8Center of Pediatrics and Neonatology, Asklepios Clinic Sankt Augustin, Sankt Augustin, Germany

    Background/Purpose: Long-term surveillance of biologic drugs is particularly important in pediatric patients(pts). Since 2001, the German Biologics JIA Registry (BIKER) is allowing to follow up…
  • Abstract Number: 364 • 2017 ACR/ARHP Annual Meeting

    Canakinumab First or Second Choice in Systemic Juvenile Idiopathic Arthritis – Experience from Clinical Practice

    Gerd Horneff1, Ivan Foeldvari2, Klaus Tenbrock3, K Minden4 and Jasmin B. Kuemmerle-Deschner5, 1Asklepios Clinic, Sankt Augustin, Germany, 2Kinder- und Jugenrheumatologie, Hamburger Zentrum Kinder-und Jugendrheumatologie, Hamburg, Germany, 3University Aachen, Aachen, Germany, 4Charité – University of Medicine Berlin, Berlin, Germany, 5Pediatrics, University Hospital Tübingen, Tübingen, Germany

    Background/Purpose: Canakinumab (CAN) has demonstrated its efficacy and safety in systemic juvenile idiopathic arthritis in clinical trials. We report on the experience with CAN in…
  • Abstract Number: 1885 • 2017 ACR/ARHP Annual Meeting

    Epidemiology of Hospitalized Adult Onset Still’s Disease in United States

    Bella Y. Mehta1, William Briggs2 and Petros Efthimiou3, 1Rheumatology, Hospital for Special Surgery/Weill Cornell Medicine/Mailman School of Public Health, New York, NY, 2New York Presbyterian/ Brooklyn Methodist Hospital, New York, NY, 3Medicine/Rheumatology, New York University School of Medicine/NYU Langone Health, New York, NY

    Background/Purpose: There is a dearth of epidemiological studies on Adult Onset Still’s Disease (AOSD) and no consensus on its incidence and prevalence. Most studies report…
  • Abstract Number: 2109 • 2017 ACR/ARHP Annual Meeting

    Hyperferritinemic Syndrome in a General Hospital

    Ignacio Javier Gandino1, Florencia Pierini2, Jose Maximiliano Martinez P3, Santiago Ruta2, Marina Scolnik2 and Enrique R Soriano4, 1Reumatologia, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina, 2Rheumatology Unit, Internal Medicine Service, Hospital Italiano de Buenos Aires, CABA, Argentina, 3Rheumatology, Internal Medicine Service, Rheumatology Unit, Internal Medicine Service, Hospital Italiano de Buenos Aires, Instituto Universitario Hospital Italiano de Buenos Aires, and Fundacion PM Catoggio, Buenos Aires, Argentina, 4Argentina, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

    Background/Purpose: Hyperferritinemia is associated with inflammatory conditions, such as rheumatologic diseases. Objectives: To determine which diseases are associated with hyperferritinemia in a tertiary hospital; to…
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