Abstracts tagged "Systemic JIA"

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…
Abstract Number: 2325 • 2017 ACR/ARHP Annual Meeting
Extensive Serum Cytokine Analysis in Patients with Macrophage Activation Syndrome Complicating Systemic Juvenile Idiopathic Arthritis
Mao Mizuta¹, Masaki Shimizu¹, Natsumi Inoue¹, Kazuko Kasai², Yasuo Nakagishi³ and Akihiro Yachie⁴, ¹Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University, Kanazawa, Japan, ²Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, kobe, Japan, ³Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Japan, ⁴Department of Pediatrics, Graduate School of Medical Sciences, Kanazawa University,, Kanazawa, Japan
Background/Purpose: The pathogenesis of Macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA) is still unknown, but overproduction of proinflammatory cytokines from activated T…
Abstract Number: 2331 • 2017 ACR/ARHP Annual Meeting
Next Generation Sequencing Analysis of Familial Haemophagocytic Lymphohistiocytosis (HLH) Related Genes in Macrophage Activation Syndrome (MAS) and Secondary HLH (sHLH)
Chiara Passarelli¹, Manuela Pardeo², Ivan Caiello³, Elisa Pisaneschi¹, Antonio Novelli¹, Fabrizio De Benedetti⁴ and Claudia Bracaglia², ¹Unit of Medical Genetics, Laboratory of Cytogenetics and Molecular Genetics, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ²Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁴Division of Rheumatology, IRCCS Bambino Gesù Children's Hospital, Rome, Rome, Italy
Background/Purpose: Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease, particularly of systemic JIA (sJIA). It is currently classified among the secondary forms…
Abstract Number: 2332 • 2017 ACR/ARHP Annual Meeting
Biomarkers for the Diagnosis and the Identification of Risk of Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (sJIA)
Claudia Bracaglia¹, Denise Pires Marafon², Ivan Caiello³, Kathy de Graaf⁴, Maria Ballabio⁴, Walter Ferlin⁴, Sergio Davì⁵, Grant Schulert⁶, Angelo Ravelli⁷, Alexei A. Grom⁸, Robert Nelson⁴, Cristina de Min⁴ and Fabrizio De Benedetti², ¹Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ²Division of Rheumatology, IRCCS Bambino Gesù Children's Hospital, Rome, Rome, Italy, ³Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, Rome, Italy, ⁴NovImmune S.A., Geneva, Switzerland, ⁵University of Genova, IRCCS Istituto Giannina Gaslini, Genoa, Italy, ⁶Pediatric Rheumatology, Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, ⁷University of Genova, IRCCS Istituto Giannina Gaslini, Genova, Italy, ⁸Division of Pediatric Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, United States Minor Outlying Islands
Background/Purpose: We have recently reported high levels of IFNγ and of the IFNγ-related chemokines, (CXCL9 and CXCL10) in patients with MAS (1). Methods: Circulating levels…
Abstract Number: 42 • 2017 Pediatric Rheumatology Symposium
Evaluation of a Dosing Regimen for Tocilizumab in Patients Younger Than Two Years of Age With Systemic Juvenile Idiopathic Arthritis
Navita L. Mallalieu¹, Joy Hsu¹, Karen Wang¹, Sunethra Wimalasundera², Chris Wells², Inmaculada Calvo Penades³, Rubén J. Cuttica⁴, Hans-Iko Huppertz⁵, Rik Joos⁶, Yukiko Kimura⁷, Diana Milojevic⁸, Margalit Rosenkranz⁹, Kenneth Schikler¹⁰, Tamas Constantin¹¹ and Carine Wouters¹², ¹Roche Innovation Center, New York, NY, ²Roche Products Ltd., Welwyn Garden City, United Kingdom, ³Hospital Universitario y Polytécnico La Fe, Valencia, Spain, ⁴Hospital General de Niños Pedro de Elizalde, Buenes Aires, Argentina, ⁵Professor Hess Children's Hospital, Bremen, Germany, ⁶ZNA Jan Palfijn Antwerpen, Antwerp, Belgium, ⁷Hackensack University Medical Center, Hackensack, NJ, ⁸Tufts Medical Center, Boston, MA, ⁹Children's Hospital of Pittsburgh of UPMC, Pittsburgh, PA, ¹⁰University of Louisville Hospital, Louisville, KY, ¹¹Semmelweis University, Budapest, Hungary, ¹²University Hospital Gasthuisberg, Leuven, Belgium
Background/Purpose: Tocilizumab (TCZ) is approved for the treatment of systemic juvenile idiopathic arthritis (sJIA) based on clinical trials in patients ≥2 years of age. This…
Abstract Number: 130 • 2017 Pediatric Rheumatology Symposium
Validation of MRP8/14 serum levels as biomarker for the diagnosis of systemic juvenile idiopathic arthritis in fever of unknown origin
Dirk Holzinger¹, Carolin Pretzer², Maria Miranda-Garcia^2,3, Hans Huppertz⁴, Gerd Horneff⁵, Johannes Peter Haas⁶, Gerd Ganser⁷, Jasmin B. Kuemmerle-Deschner⁸, Michael Frosch⁹, Johannes Roth¹⁰ and Dirk Foell², ¹Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ²Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ³Federal Institute for Vaccines and Biomedicines, Paul-Ehrlich-Institute, Langen, Germany, ⁴Prof Hess Children’s Hospital Bremen, Bremen, Germany, ⁵Department of Pediatrics, Asklepios Clinics St. Augustin, Sankt Augustin, Germany, ⁶Centre for Pediatric Rheumatology Garmisch-Partenkirchen, Garmisch-Partenkirchen, Germany, ⁷Pediatric Rheumatology, Sankt Josef Stift Sendenhorst, Sendenhorst, Germany, ⁸Pediatrics, University Hospital Tuebingen, Tuebingen, Germany, ⁹Pediatric Pain Centre,, Children's and Adolescents' Hospital Datteln, Datteln, Germany, ¹⁰Institute of Immunology, University of Muenster, Muenster, Germany
Background/Purpose: The differential diagnosis of fever of unknown origin (FUO) is a major challenge in pediatrics especially for differentiation of systemic-onset juvenile idiopathic arthritis (SJIA)…
Abstract Number: 70 • 2017 Pediatric Rheumatology Symposium
Impact of Systemic Juvenile Idiopathic Arthritis/Still’s Disease on Adolescents as Evidenced Through Social Media Posting
Renee F Modica¹, Kathleen G Lomax², Pamela Batzel³, Armelle Cassanas³ and Melissa E Elder¹, ¹Department of Pediatrics, University of Florida, Gainesville, FL, ²Novartis Pharmaceuticals Corporation, East Hanover, NJ, ³Treato, Princeton, NJ
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA)/Still’s disease is a rare form of chronic arthritis in pediatrics. The patient perspective of living with the disease is…
Abstract Number: 76 • 2017 Pediatric Rheumatology Symposium
Applying 2016 MAS Criteria to Systemic onset Juvenile Idiopathic Arthritis Patients with Diagnosis of Macrophage Activation Syndrome
Ezgi Baris^1,2, Edwin Anderson³ and Fatma Dedeoglu¹, ¹Division of Immunology, Rheumatology Program, Boston Children's Hospital, Harvard Medical School, Boston, MA, ²Pediatrics, Marmara University, Istanbul, Turkey, ³Division of Immunology, Boston Children's Hospital, Boston, MA
Background/Purpose: Macrophage activation syndrome (MAS) is the result of uncontrolled systemic inflammation, which can sometimes complicate systemic onset juvenile idiopathic arthritis (SoJIA). MAS classification criteria…
Abstract Number: 71 • 2017 Pediatric Rheumatology Symposium
Consensus-based diagnostic approach to systemic juvenile idiopathic arthritis in Germany
Claas Hinze¹, Dirk Holzinger¹, Elke Lainka², Johannes Peter Haas³, Tilmann Kallinich⁴, Ulrich Neudorf², Helmut Wittkowski¹, Gerd Horneff⁵, Dirk Foell⁶ and PRO-KIND study group on SJIA, ¹Department of Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany, ²University of Essen, Essen, Germany, ³Center for Pediatric and Adolescent Rheumatology Garmisch-Partenkirchen, Garmisch-Partenkirchen, Germany, ⁴Charite, University Medicine Berlin, Berlin, Germany, ⁵Asklepios Klinik Sankt Augustin GmbH, Sankt Augustin, Germany, ⁶Pediatric Rheumatology and Immunology, University of Muenster, Muenster, Germany
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is currently classified by the International League of Associations for Rheumatology (ILAR) classification criteria. It is characterized by severe…

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