Abstracts tagged "Systemic JIA"

Abstract Number: 1416 • 2018 ACR/ARHP Annual Meeting
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Simone Carbogno¹, Denise Pires Marafon², Giulia Marucci³, Manuela Pardeo³, Antonella Insalaco³, Virginia Messia³, Rebecca Nicolai³, Fabrizio De Benedetti⁴ and Claudia Bracaglia³, ¹University of Milan, Milan, Italy, ²Pediatric Unit, Fondazione IRCCS Ca’ Grande Ospedale Maggiore Policlinico, Milan, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁴IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of rheumatic diseases in childhood, particularly of systemic Juvenile Idiopathic Arthritis (sJIA), occurring in approximately…
Abstract Number: 1426 • 2018 ACR/ARHP Annual Meeting
The Utilization of S100 Proteins Testing in Pediatric Rheumatology Patients in a Tertiary Care Institution and Implications for Care
Najla Aljaberi¹, Angela Merritt², Alexei Grom¹, Grant Schulert³, Jennifer L. Huggins⁴, Michael Henrickson⁵ and Hermine I. Brunner¹, ¹Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Pediatric Rheumatology, Division of Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁴Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Cincinnati Children’s Medical Center, Cincinnati, OH
Background/Purpose: S100 proteins are calcium-binding proteins of increasing value as biomarkers in various inflammatory conditions (e.g. autoinflammatory diseases, vasculitides, inflammatory bowel disease). The two highly…
Abstract Number: 1437 • 2018 ACR/ARHP Annual Meeting
Use,Safety and Efficacy of Thalidomide from a Tertiary Level Pediatric Rheumatology Centre in India
Manjari Agarwal¹ and Sujata Sawhney², ¹Institute of Child Health, Attending Consultant, New Delhi, India, ²Paediatric rheumatology, Senior Consultant, New Delhi, India
Background/Purpose: Thalidomide is an effective agent for several pediatric rheumatic diseases: SOJIA, Behcet's disease and recalcitrant skin disease in cSLE to name a few. Systemic…
Abstract Number: 2864 • 2018 ACR/ARHP Annual Meeting
Identification of Optimal Subcutaneous Doses of Tocilizumab in Children with Systemic Juvenile Idiopathic Arthritis
Hermine I. Brunner¹, Nicola Ruperto², Daniel J Lovell¹, Gerd Horneff³, María Luz Gámir-Gámir⁴, Markus Hufnagel⁵, Joy Hsu⁶, Min Bao⁶, Wendy Douglass⁷, Navita L. Mallalieu⁶, Chris Wells⁷, Christopher M. Mela⁸ and Fabrizio De Benedetti⁹, ¹Pediatric Rheumatology Collaborative Study Group (PRCSG), Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Pediatric Rheumatology International Trial Organization (PRINTO), Istituto Giannina Gaslini Pediatria II-Reumatologia, Genoa, Italy, ³Asklepios Clinic Sankt Augustin, and University Hospital of Cologne, Cologne, Germany, ⁴Hospital Ramon y Cajal Unidad de Reumatologia Pediatrica, Madrid, Spain, ⁵Department of Pediatrics and Adolescent Medicine, University Medical Center, Medical Faculty, University of Freiburg, Freiburg, Germany, ⁶Roche Innovation Center, New York, NY, ⁷Roche Products Ltd., Welwyn Garden City, United Kingdom, ⁸Roche Products Ltd, Welwyn Garden City, United Kingdom, ⁹IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy
Background/Purpose: Efficacy and safety of intravenous (IV) tocilizumab (TCZ) were shown in patients (pts) with systemic JIA (sJIA) in the phase 3 TENDER study.1 This…
Abstract Number: 2865 • 2018 ACR/ARHP Annual Meeting
Canakinumab, on a Reduced Dose or a Prolonged Dose Interval without Concomitant Corticosteroids and Methotrexate, Maintains Efficacy in Systemic Juvenile Idiopathic Arthritis Patients in Clinical Remission
Pierre Quartier¹, Ekaterina Alexeeva², Carine Wouters³, Inmaculada Calvo⁴, Tilmann Kallinich⁵, Bo Magnusson⁶, Nico Wulffraat⁷, Xiaoling Wei⁸, Alan Slade⁹, Ken Abrams⁹ and Alberto Martini¹⁰, ¹AP-HP, Institut des Maladies Génétiques (IMAGINE), and Université Paris-Descartes, Necker-Enfants Malades Hospital, Paris, France, ²National Medical Research Center of Children's Health and Sechenov First Moscow State Medical University of the Ministry of Health of the Russian Federation, Moscow, Russian Federation, ³Gasthuisberg University Hospital, Leuven, Belgium, ⁴Hospital Universitario La Fe, Valencia, Spain, ⁵Charité Berlin Campus Virchow, Berlin, Germany, ⁶Karolinska University Hospital, Stockholm, Sweden, ⁷University Medical Center Utrecht, Utrecht, Netherlands, ⁸China Novartis Institutes for Biomedical Research Co., Ltd, Beijing, China, ⁹Novartis Pharmaceuticals Corporation, East Hanover, NJ, ¹⁰Universita di Genova Pediatria II, Genova, Italy
Background/Purpose: Treatment with canakinumab (CAN), a selective, human anti-IL-1β monoclonal antibody, has shown sustained therapeutic effect along with corticosteroid dose reduction/discontinuation in patients with systemic…
Abstract Number: 2917 • 2018 ACR/ARHP Annual Meeting
Multiplex Serum Biomarker Analysis before and during Therapy with Canakinumab in Patients with Systemic Juvenile Idiopathic Arthritis
Tanja Hinze¹, Christoph Kessel¹, Claas Hinze¹, Julia Seibert², Hermann Gram² and Dirk Foell³, ¹Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ²Novartis, Basel, Switzerland, ³Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany
Background/Purpose: Canakinumab (CAN), a monoclonal anti-interleukin (IL)-1β antibody, is approved for the treatment of systemic juvenile idiopathic arthritis (SJIA). CAN-treated patients with SJIA showed a…
Abstract Number: 923 • 2018 ACR/ARHP Annual Meeting
Neutrophils from Children with Systemic JIA Exhibit Persistent Proinflammatory Activation Despite Long-Standing Clinically Inactive Disease
Rachel Brown¹, Maggie Henderlight¹, Thuy Do¹, Shima Yasin², Monica DeLay¹, Alexei A. Grom^3,4 and Grant Schulert^2,5, ¹Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Rheumatology, Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ³Pediatrics, University of Cincinnati, CINCINNATI, OH, ⁴Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁵Pediatrics, Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a chronic childhood arthropathy with features of autoinflammation. New-onset SJIA is associated with expansion and activation of neutrophils…
Abstract Number: 927 • 2018 ACR/ARHP Annual Meeting
The Reprogramming of Regulatory T Cells to a Th17 Phenotype in Systemic Juvenile Idiopathic Arthritis
Lauren A. Henderson¹, Kacie Hoyt², Jennifer P. Nguyen³, Pui Lee¹, Deepak Rao⁴, Anna Helena Jonsson⁵, Erin Janssen¹, Fatma Dedeoglu¹, Melissa M. Hazen¹, Mindy S. Lo¹, Esra Meidan¹, Mary Beth Son¹, Robert Sundel¹, Matthew L. Stoll⁶, Chad Nusbaum⁷, James A. Lederer⁸, Talal A. Chatila⁹ and Peter A. Nigrovic^1,5, ¹Division of Immunology, Boston Children's Hospital, Harvard Medical School, Boston, MA, ²Division of Immunology, Boston Children's Hospital, Boston, MA, ³Department of Surgery, Brigham and Women's Hospital, Boston, MA, ⁴Human Immunology Center, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁵Division of Rheumatology, Immunology, and Allergy, Brigham and Women's Hospital, Harvard Medical School, Boston, MA, ⁶Pediatric Rheumatology, University of Alabama at Birmingham, Birmingham, AL, ⁷Broad Technology Labs, Broad Institute, Cambridge, MA, ⁸Department of Surgery, Brigham and Women's Hospital and Harvard Medical School, Boston, MA, ⁹Division of Immunology, Boston Children's Hospital, Harvard Medical School, BOSTON, MA
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever and rash at disease onset, which are mediated in part by IL-1β and IL-6. In…
Abstract Number: 365 • 2017 ACR/ARHP Annual Meeting
Canakinumab Treatment in Patients with Still’s Disease: A Pooled Analysis of Systemic Juvenile Idiopathic Arthritis Data By Age Groups
Eugen Feist¹, Pierre Quartier², Bruno Fautrel³, Rayfel Schneider⁴, Paolo Sfriso⁵, Petros Efthimiou⁶, Luca Cantarini⁷, Karine Lheritier⁸, Karolynn Leon⁹ and Antonio Speziale⁸, ¹Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin, Berlin, Germany, ²Necker-Enfants Malades Hospital, Paris, France, ³UPMC University Paris 06, Pitié-Salpétrière Hospital, Paris, France, ⁴University of Toronto and The Hospital for Sick Children, Toronto, ON, Canada, ⁵University of Padova, Padova, Italy, ⁶Medicine/Rheumatology, New York University School of Medicine/NYU Langone Health, New York, NY, ⁷University of Siena, Siena, Italy, ⁸Novartis Pharma AG, Basel, Switzerland, ⁹Novartis Pharmaceuticals Corporation, East Hanover, NJ
Background/Purpose: Still’s disease presents in pediatric and adult patients as a disease continuum with similar symptoms and pathophysiology.1,2 The objective of this analysis was to…
Abstract Number: 366 • 2017 ACR/ARHP Annual Meeting
Short-Term Outcomes in Patients with Systemic-Onset Juvenile Idiopathic Arthritis Treated with Either Tocilizumab or Anakinra in a Real-World Setting in the United Kingdom
Lianne Kearsley-Fleet¹, Diederik De Cock¹, Eileen Baildam², Michael W. Beresford³, Helen E. Foster⁴, Taunton R. Southwood⁵, Wendy Thomson^6,7 and Kimme L. Hyrich^1,6, ¹Arthritis Research UK Centre for Epidemiology, University of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom, ²Clinical Academic Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Liverpool, United Kingdom, ³Alder Hey Children's NHS Foundation Trust Hospital, Institute of Translational Medicine (Child Health), University of Liverpool, Liverpool, United Kingdom, ⁴Institute of Cellular Medicine and Paediatric Rheumatology, Newcastle University and Great North Children's Hospital, Newcastle Upon Tyne, United Kingdom, ⁵Institute of Child Health, University of Birmingham and Birmingham Children's Hospital, Birmingham, United Kingdom, ⁶National Institute of Health Research Manchester Musculoskeletal Biomedical Research Centre, Central Manchester NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom, ⁷Arthritis Research UK Centre for Genetics and Genomics, University of Manchester, Manchester Academic Health Science Centre, Manchester, United Kingdom
Background/Purpose: Juvenile idiopathic arthritis (JIA) comprises 7 ILAR categories, but systemic-onset JIA (sJIA) appears to be distinct in genetic background and pathogenesis from the other…
Abstract Number: 370 • 2017 ACR/ARHP Annual Meeting
Validation of 2016 ACR/EULAR Classification Criteria of Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis in Japanese Patients
Masaki Shimizu¹, Mao Mizuta¹, Takahiro Yasumi², Naomi Iwata³, Yuka Okura⁴, Noriko Kinjo⁵, Hiroaki Umebayashi⁶, Tomohiro Kubota⁷, Yasuo Nakagishi⁸, Kenichi Nishimura⁹, Masato Yashiro¹⁰, Junko Yasumura¹¹, Kazuko Yamazaki¹², Hiroyuki Wakiguchi¹³, Nami Okamoto¹⁴ and Masaaki Mori¹⁵, ¹Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan, ²Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan, ³Department of Immunology and Infectious Diseases, Aichi Children’s Health and Medical Center, Obu, Japan, ⁴Department of Pediatrics, KKR Sapporo Medical Center, Sapporo, Japan, ⁵Department of Pediatrics, Faculty of medicine, University of the Ryukyus, Nakagami-gun, Japan, ⁶Department of Rheumatics, Miyagi Children’s Hospital, Sendai, Japan, ⁷Department of Pediatrics, Kagoshima University Graduate School of Medical and Dental Sciences, Kagoshima, Japan, Kagoshima, Japan, ⁸Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan, ⁹Department of Pediatrics, Yokohama City University Graduate School of Medicine, Yokohama, Japan, ¹⁰Department of Pediatrics, Okayama University Hospital, Okayama, Japan, ¹¹Department of Pediatrics, Hiroshima University Graduate School of Biomedical & Health Sciences, Hiroshima, Japan, ¹²Department of Pediatrics, Saitama Medical Center, Saitama Medical University, Kawagoe, Japan, ¹³Department of Pediatrics, Yamaguchi University Graduate School of Medicine, Ube, Japan, ¹⁴Department of Pediatrics, Osaka Medical College, Takatsuki, Japan, ¹⁵Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan
Background/Purpose: To validate the 2016 ACR/EULAR classification criteria of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) in Japanese patients. Methods: A combination…
Abstract Number: 940 • 2017 ACR/ARHP Annual Meeting
IL1RN Variation Is Associated with Systemic Juvenile Idiopathic Arthritis and Predicts Non-Response to Anakinra Treatment
Emily Shuldiner¹, Victoria Arthur¹, Anne Hinks², Patricia Woo³, Wendy Thomson², Elaine F. Remmers⁴ and Michael J. Ombrello¹, ¹Translational Genetics and Genomics Unit, NIAMS, NIH, Bethesda, MD, ²Arthritis Research UK Centre for Genetics and Genomics, Centre for Musculoskeletal Research, Manchester Academic Health Sciences Centre, The University of Manchester, Manchester, United Kingdom, ³University College London, London, United Kingdom, ⁴Genetics and Genomics Branch, NIH, NIAMS, Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood inflammatory disease whose pathophysiology is poorly understood. sJIA is phenotypically heterogeneous with variable manifestations and responses…
Abstract Number: 1895 • 2017 ACR/ARHP Annual Meeting
Serum Interleukin 18 As a Biomarker for Systemic Juvenile Idiopathic Arthritis and Macrophage Activation Syndrome and Use of Recombinant Human IL-18 BP in a Patient with Refractory Disease
Shima Yasin¹, Rachel Brown¹, Ndate Fall², Krista Solomon¹, Scott Canna³, Charlotte Girard⁴, Cem Gabay⁵, Eduardo Schiffrin⁶, Andrew Sleight⁶, Alexei A. Grom⁷ and Grant Schulert⁸, ¹Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Division of Rheumatology, Cincinnati Children's Hospital, Cincinnati, OH, ³NIAMS, National Institutes of Health, Bethesda, MD, ⁴Division of Rheumatology, Department of Internal Medicine Specialties, University Hospital of Geneva, Geneva, Switzerland, ⁵SCQM, Geneva, Switzerland, Geneva, Switzerland, ⁶AB2 Bio, Lausanne, Switzerland, ⁷Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Minor Outlying Islands, ⁸Pediatric Rheumatology, Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis is an autoinflammatory childhood arthritis with prominent innate immune activity. Macrophage activation syndrome is a severe and potentially fatal complication…
Abstract Number: 1896 • 2017 ACR/ARHP Annual Meeting
IL-18 As a Diagnostic Biomarker, Differentiating Systemic JIA from Acute Leukaemia, Severe Bacterial Infections and Other Auto-Immune Disorders
Arjen Leek¹, Nienke Ter Haar², Valerie De Haas³, Ayman El Idrissi¹, Judith Wienke¹, Sytze de Roock⁴, Dirk Holzinger⁵, Wilco de Jager⁶, Jorg van Loosdregt⁷ and Sebastiaan Vastert^4,8, ¹Pediatric Rheumatology and Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ²Department of Pediatric Rheumatology and immunology, University Medical Center Utrecht, Utrecht, Netherlands, ³DCOG Laboratory, SKION, Den Haag, Netherlands, ⁴Laboratory of Translational Immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁵Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ⁶Dept Immunology, UMC Utrecht, Utrecht, Netherlands, ⁷Laboratory for Translational immunology, University Medical Center Utrecht, Utrecht, Netherlands, ⁸Division of Pediatric Rheumatology, University Medical Center Utrecht, Utrecht, Netherlands
Background/Purpose: Systemic onset Juvenile Idiopathic Arthritis (sJIA) is a disease characterized by systemic inflammation in addition to arthritis and it’s diagnosis currently still depends on…
Abstract Number: 2324 • 2017 ACR/ARHP Annual Meeting
Single Cell RNA-Sequencing of Bone Marrow Macrophages Identifies a Distinct Subpopulation in Systemic JIA with Features of Interferon Response, Endocytic Vesicles and Phagocytosis
Grant Schulert¹, Nathan Salomonis², Sherry Thornton³ and Alexei A. Grom⁴, ¹Pediatric Rheumatology, Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ²Division of Biomedical Informatics, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁴Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Minor Outlying Islands
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of systemic juvenile idiopathic arthritis (SJIA), characterized by activation and expansion of cytolytic lymphocytes and macrophages…