Abstracts tagged "Systemic JIA"

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: 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: 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…
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…

All abstracts accepted to ACR Convergence are under media embargo once the ACR has notified presenters of their abstract’s acceptance. They may be presented at other meetings or published as manuscripts after this time but should not be discussed in non-scholarly venues or outlets. The following embargo policies are strictly enforced by the ACR.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM ET on November 14, 2024. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].