ACR Meeting Abstracts

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Abstracts tagged "Still’s disease"

  • Abstract Number: 0826 • ACR Convergence 2022

    Treatment Pattern and Changes in Oral Glucocorticoid Dose After Tocilizumab Treatment in Patients with Adult Still’s Disease: An Analysis of a Japanese Claims Database

    Yuko Kaneko1, Hideto Kameda2, Kei Ikeda3, Katsuhisa Yamashita4, Ryoto Ozaki5 and Yoshiya Tanaka6, 1Keio University School of Medicine, Tokyo, Japan, 2Toho University, Tokyo, Japan, 3Chiba University Hospital, Chiba, Japan, 4Chugai Pharmaceutical Co., Ltd., Chuo-ku, Tokyo, Japan, 5Chugai Pharmaceutical Co. Ltd., Tokyo, Japan, 6University of Occupational and Environmental Health, Kitakyusyu Fukuoka, Japan

    Background/Purpose: Adult Still's disease (ASD), including adult-onset Still's disease and carry-over systemic juvenile idiopathic arthritis (sJIA), is treated with glucocorticoids (GC) with or without immunosuppressive…
  • Abstract Number: 0829 • ACR Convergence 2022

    Adult Onset Still’s Disease: A Retrospective Study of Objective Manifestations and Outcomes

    Caroline Bui1, Tim Collins2, Kumar Venkat3, Napatkamon Ayutyanont2, Kim Vickery4 and Scott Kubomoto2, 1Riverside Community Hospital/University of California Riverside, Riverside, CA, 2Riverside Community Hospital/University of California Riverside, Riverside, 3University of California Riverside/Riverside Community Hospital, Riverside, 4HCA Healthcare, Riverside

    Background/Purpose: Adult Onset Still's Disease (AOSD) is a complex and rare inflammatory disease with life threatening complications. Due to its rarity and wide ranging clinical…
  • Abstract Number: 0863 • ACR Convergence 2022

    Effect of Glucocorticoids on Patient Reported Outcomes in Patients Started on a Biologic Consensus Treatment Plan for the ‘First Line Options for Systemic JIA Treatment’ (FROST) Study

    Karen James1, George Tomlinson2, Tim Beukelman3, Laura Schanberg4, Anne Dennos5, VIncent Del Gaizo6, Marian Jelinek7, Erin Pfeifer8, Shalini Mohan9 and Yukiko Kimura10, 1University of Utah, Salt Lake City, UT, 2University of Toronto, Toronto, ON, Canada, 3Childhood Arthritis and Rheumatology Research Alliance (CARRA), Birmingham, AL, 4Duke University Medical Center, Durham, NC, 5Duke University, Durham, NC, 6Childhood Arthritis and Rheumatology Research Alliance (CARRA), Durham, NC, 7Childhood Arthritis and Rheumatology Research Alliance (CARRA), Duke, 8Genentech, Englewood, NJ, 9Genentech, San Diego, 10Hackensack Meridian Health, New York, NY

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a systemic autoinflammatory disease characterized by high fevers, rash and arthritis. Current treatment regimens often involve biologic (anti-IL-1…
  • Abstract Number: 0864 • ACR Convergence 2022

    Baseline Clinical and Laboratory Features of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Systemic Juvenile Idiopathic Arthritis-Associated Lung Disease (SJIA-LD) Cohort

    Esraa Eloseily1, Min-Lee Chang2, MaryEllen Riordan3, Alan Russell4, Marc Natter2, Yukiko Kimura5 and Grant Schulert6, 1Division of Pediatric Rheumatology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 2Boston Children's Hospital, Boston, MA, 3Hackensack Meridian Health, Hackensack, NJ, 4Duke Clinical Research Institute, Duke, NC, 5Hackensack Meridian Health, New York, NY, 6Cincinnati Children's Hospital Medical Center, Cincinnati, OH

    Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) associated lung disease (SJIA-LD) is an emerging and life threatening clinical problem, and currently affects as many as 1…
  • Abstract Number: 0867 • ACR Convergence 2022

    Regulatory Haplotype of CXCR4 Is Associated with sJIA and Corelates with Enhanced Neutrophil and CD14+ Monocyte Migration

    Hiroto Nakano1, Emily Shuldiner2, Anne Hinks3, Marc Sudman4, Elaine Remmers5, Colleen Satorius6, Elizabeth Schmitz1, Victoria Arthur7, Patricia Woo8, Alexei Grom9, Dirk Foell10, John Bohnsack11, Marco Gattorno12, Seza Ozen13, Sampath Prahalad14, Rae Yeung15, Elizabeth Mellins2, Sheila Oliveira16, Jordi Antón17, Claudio Len18, Carol Lake19, Ly-Lan Bergeron20, Michelle Millwood21, Estefania de los santos21, Mariana Correia Marques22, Juvenile Arthritis Consortium for the Immunochip23, The Genomic Ascertainment Cohort Investigators24, INCHARGE Consortium25, Carl Langefeld26, Susan Thompson27, Wendy Thomson28 and Michael Ombrello1, 1National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 2Stanford University, Stanford, CA, 3The University of Manchester, Manchester, United Kingdom, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5National Human Genome Research Institute, Bethesda, MD, 6NHGRI, NIH, Bethesda, MD, 7Boston Children's Hospital, Boston, MA, 8University College London, London, United Kingdom, 9Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 10University Hospital Münster, Münster, Germany, 11University of Utah, Salt Lake City, UT, 12Pediatric Clinic and Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy, 13Hacettepe University Faculty of Medicine, Ankara, Turkey, 14Emory + Children's Pediatric Institute, Atlanta, GA, 15The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, 16Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, 17Pediatric Rheumatology Department. Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Spain, 18Universidade Federal de São Paulo, São Paulo, Brazil, 19NIH, Gaithersburg, MD, 20NIH/NIAMS, Vienna, VA, 21NIAMS, NIH, Bethesda, MD, 22National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, 23Juvenile Arthritis Consortium for the Immunochip, Bethesda, MD, 24The Genomic Ascertainment Cohort Investigators, Bethesda, MD, 25International Childhood Arthritis Genetics Consortium, Bethesda, MD, 26Wake Forest School of Medicine, Winston Salem, NC, 27Cincinnati Children's Hospital Medical Center/Univ of Cincinnati College of Medicine, Blue Ash, OH, 28Manchester Academic Health Science Centre, Manchester, United Kingdom

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare inflammatory disease that causes spiking fever, skin rash, chronic arthritis, and inflammation of the heart and…
  • Abstract Number: 0195 • ACR Convergence 2021

    JAK Inhibitors in Refractory Adult and Childhood-Onset Still’s Disease

    Louise GILLARD1, Stéphane Mitrovic2, Jacques Pouchot3, Fleur Cohen1, Martin Michaud4, Heloise Reumaux5, Isabelle Kone-Paut6 and Bruno Fautrel7, 1Pitié Salpêtrière Hospital, Paris, France, 2Pitié Salpêtrière Hospital - Institut Mutaliste Montsouris, Paris, France, 3AP-HP, RUEIL-MALMAISON, France, 4Joseph Ducuing Hospital, Toulouse, France, 5Jeanne de Flandre Hospital, Lille, France, 6APHP, Bicetre hospital, Le Kremlin bictre, France, 7Pitié Salpêtrière Hospital, APHP, Sorbonne Université, Paris, France

    Background/Purpose: Excessive and inappropriate production of pro-inflammatory cytokines such as interleukin IL-1, IL-6 or IL-18, is a pathogenic cornerstone in adult and childhood onset Still’s…
  • Abstract Number: 0242 • ACR Convergence 2021

    FiRst Line Options for Systemic JIA Treatment (FROST): Results from a Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry Consensus Treatment Plan Observational Study

    Timothy Beukelman1, George Tomlinson2, Peter Nigrovic3, Anne Dennos4, Vincent Del Gaizo5, Mary Ellen Riordan6, Laura Schanberg7, Shalini Mohan8, Erin Pfeifer9 and Yukiko Kimura10, 1University of Alabama at Birmingham, Birmingham, AL, 2University of Toronto, Toronto, ON, Canada, 3Boston Children's Hospital, Brookline, MA, 4Duke University, Durham, NC, 5Childhood Arthritis & Rheumatology Research Alliance (CARRA), Whitehouse Station, NJ, 6Hackensack University Medical Center, Westwood, NJ, 7Duke University Medical Center, Durham, NC, 8Genentech, Inc., South San Francisco, CA, 9Genentech, Inc., Englewood, CO, 10Hackensack University Medical Center, New York, NY

    Background/Purpose: The optimal initial treatment for systemic juvenile idiopathic arthritis (sJIA) is unclear. To further study the initial treatment of sJIA, the Childhood Arthritis and…
  • Abstract Number: 1100 • ACR Convergence 2021

    Pulmonary Arterial Hypertension in Adult-Onset Still’s Disease: A Case Series of 13 Patients

    Stéphane Mitrovic1, athénaïs Boucly2, Estibaliz Lazaro3, Nicolas SCHLEINITZ4, Coralie Bloch-Queyrat5, Christine Christides6, Jacques Pouchot7, marc humbert2, David Montani2, laurent savale2, Xavier Jaïs2, Olivier Sitbon2 and Bruno Fautrel8, 1Pitié Salpêtrière Hospital - Institut Mutaliste Montsouris, Paris, France, 2Pneumology Department, Bicêtre Hospital, Assistance Publique – Hôpitaux de Paris, Kremlin-Bicêtre, France, 3Internal Medicine Department, CHU Bordeaux, Pessac, France, 4Internal Medicine Department, Hôpital Timone, APHM, Marseille, France, 5Internal Medicine Department, Avicennes Hospital, APHP, Bobigny, France, 6Internal Medicine Department, CH Avignon, Avignon, France, 7AP-HP, RUEIL-MALMAISON, France, 8Pitié Salpêtrière Hospital, APHP, Sorbonne Université, Paris, France

    Background/Purpose: Pulmonary Arterial Hypertension (PAH) is a rare but potentially fatal complication of Adult-Onset Still's Disease (AOSD). To date, only isolated observations have been published.…
  • Abstract Number: 1548 • ACR Convergence 2021

    Q Fever as a Mimicker of Rheumatologic Conditions: A Case Series from Two Tertiary Care Academic Centers in Southern California

    Manushi Aggarwal and Marven Cabling, Loma Linda University Health, Redlands, CA

    Background/Purpose: Q fever, an endemic disease in Southern California, is a zoonosis caused by Coxiella burnetii. The infection can present with multiple non-specific acute and chronic manifestations including fever, headache,…
  • Abstract Number: 1629 • ACR Convergence 2021

    Effect of Drug Withdrawal on Interleukin-1 or Interleukin-6 Inhibitor Associated Diffuse Lung Disease

    Vivian Saper1, Sampath Prahalad2, Scott Canna3, Rabheh Abdul-Aziz4, Marcela Alvarez5, Catherine Bingham6, Brigitte Bader-Meunier7, Imelda Balboni8, Roberta Berard9, Roxana Bolaria10, Alexis Boneparth11, Alicia Casey12, Elaine Cassidy13, Joyce C. Chang14, Michal Cidon15, Kathleen Collins16, Aileen M. Dickenson17, Graciela Espada5, Martha Fishman12, Elaine Flanagan18, Timothy Hahn19, Ankur K. Jindal20, Ozgur Kasapcopur21, Marisa Klein-Gitelman22, Timothy Klouda12, Mikhail Kostik23, Carol Lake24, Mariana Marques25, Michael Ombrello26, Karen Onel27, Omkar Phadke28, Angelo Ravelli29, Adam Reinhardt30, Amanda D. Robinson31, Kelly Rouster-Stevens32, Nadine Saad27, Grant Schulert33, Susan Shenoi34, Cory Stingl14, Anjali Sura35, Melissa Tesher36, Jessica Tibaldi37, Kathryn Torok38, Cathy Tsin39, Natalia Vasquez-Canizares40, D. Sofia Villacis-Nunez41, Ben Whitehead42, Holly Wobma12, Lawrence Zemel43 and Elizabeth Mellins1, 1Stanford University, Stanford, CA, 2Emory + Children's Pediatric Institute, Atlanta, GA, 3Children's Hospital of Philadelphia, Philadelphia, PA, 4University at Buffalo, Buffalo, NY, 5Hospital de Niños Dr Ricardo Gutierrez, Buenos Aires, Argentina, 6Penn State Children's Hospital, Allentown, PA, 7Pediatric Immuno-hematology, Necker Hospital, APHP, Paris, France, 8Stanford University, Palo Alto, CA, 9London Health Sciences Centre, London, ON, Canada, 10Self, Victoria, BC, Canada, 11Columbia University Medical Center, New York, NY, 12Boston Children's Hospital, Boston, MA, 13University of Pittsburgh Medical Center Medical Education, Pittsburgh, PA, 14Children's Hospital of Phildelphia, Philadelphia, PA, 15Children's Hospital Los Angeles, Los Angeles, CA, 16LeBonheur Children's Hospital, Memphis, TN, 17Children's Hospital of Los Angeles, Los Angeles, CA, 18Emory University and Children's Healthcare of Atlanta, Atlanta, GA, 19Penn State Childrens Hospital, Hershey, PA, 20Postgraduate Institute of Medical Education and Research, Chandigarh, India, 21Istanbul University-Cerrahpasa, Cerrahpasa Medical School, İstanbul, Turkey, 22Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL, 23Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia, 24National Institutes of Health, Gaithersburg, MD, 25UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, 26National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 27Hospital for Special Surgery, New York, NY, 28EMORY, Atlanta, GA, 29Istituto Giannina Gaslini, Genoa, Italy, 30Boys Town National Research Hospital, Omaha, NE, 31UPMC Children's Hospital of Pittsburgh, Pittsburgh, PA, 32Emory University/Children's Healthcare of Atlanta, Atlanta, GA, 33Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 34Seattle Children's Hospital, Seattle, WA, 35SUNY Upstate Medical University, Syracuse, NY, 36University of Chicago, Chicago, IL, 37IRCSS Istituto G. Gaslini University of Genoa, Genoa, Italy, 38University of Pittsburgh, Pittsburgh, PA, 39Stanford University, Stanford, 40Children's Hospital at Montefiore, Bronx, NY, 41Children's Healthcare of Atlanta - Emory University, Atlanta, GA, 42Queensland Children's Hospital, Brisbane, Australia, 43CCMC, Hartford, CT

    Background/Purpose: Severe delayed hypersensitivity reactions (DHR) are under-recognized in inflammatory conditions, particularly drug reaction with eosinophilia and systemic symptoms (DRESS). Previous work has shown this…
  • 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…
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