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…