ACR Meeting Abstracts

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Abstracts tagged "Still’s disease"

  • Abstract Number: 0381 • ACR Convergence 2024

    Two- and 3-Year Outcomes of the Childhood Arthritis and Rheumatology Research Alliance FROST Study of New-onset Systemic JIA Treatment

    Timothy Hahn1, George Tomlinson2, Yukiko Kimura3, Vincent Del Gaizo4, Carlos Valdes5 and Timothy Beukelman6, and for the CARRA FROST Investigators, 1Penn State Childrens Hospital, Hershey, PA, 2University of Toronto, Toronto, ON, Canada, 3Hackensack Meridian School of Medicine, New York, NY, 4Childhood Arthritis & Rheumatology Research Alliance (CARRA), Whitehouse Station, NJ, 5Genentech, Davie, FL, 6University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: The FiRst Line Options for sJIA Treatment (FROST) trial was a prospective observational study designed to compare the effectiveness of 4 Childhood Arthritis and…
  • Abstract Number: 0758 • ACR Convergence 2023

    Clinical and Biological Characteristics of Children and Adults Affected with Still’s Disease: A Systematic Review and Meta-analysis Informing the 2023 EULAR/PReS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis and Adult-onset Still’s Disease

    STEPHANE MITROVIC1, Arianna De Matteis2, Sara Bindoli3, Fabrizio De Benedetti4, Bruno Fautrel5, Loreto Carmona6 and On Behalf Of The Eular/PreS QoC011 Task Force Member7, 1Pitie Salpetriere Hospital, Sorbonne University, APHP, Paris, France, 2IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, 3Rheumatology Unit, Dept of Medicine, University of Padova, Padova, Italy, 4Bambino Gesu Children's Hospital, Division of Rheumatology, Rome, Italy, 5Sorbonne Université APHP, Paris, France, 6Instituto de Salud Musculoesquelética (InMusc), Madrid, Spain, 7EULAR/PReS QoC011 Task Force members are: Jordi Anton, Alexandre Belot, Claudia Bracaglia, Tamas Constantin, Lorenzo Dagna, Alessandro De Bartolo, Eugen Feist, Dirk Foell, Marco Gattorno, Sophie Georgin-Lavialle, Roberto Giacomelli, Alexei Grom, Yvan Jamilloux, Katarina Laskari, Calin Lazar, Francesca Minoia, Peter Nigrovic, Filipa Oliveira Ramos, Seza Ozen, Pierre Quartier-dit-Maire, Piero Ruscitti, Erdal Sag, Sinisa Savic, Marie-Elise Truchetet, Bas Vastert, Tanita Wilhelmer, Carine Wouters., Paris, France

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are part of the non-familial (sporadic) systemic inflammatory disorders and are frequently mentioned as…
  • Abstract Number: 0759 • ACR Convergence 2023

    Derivation and Validation of Four Patient Clusters in Still’s Disease, Results from GIRRCS AOSD-study Group and AIDA Network Still Disease Registry

    Piero Ruscitti1, Antonio Vitale2, Ilenia Di Cola1, Roberto giacomelli3 and Luca Cantarini4, 1University of L'Aquila, L'Aquila, Italy, 2University of Siena, Siena, Italy, 3University of Rome Campus Biomedico, Rome, Italy, 4Department of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy

    Background/Purpose: Still's disease is a rare inflammatory disorder, these patients have a highly heterogeneous disease according to age of onset, clinical presentation, presence of life-threatening…
  • Abstract Number: 0760 • ACR Convergence 2023

    Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still’s Disease Treated with Emapalumab: The REAL-HLH Study

    Carl Allen1, Shanmuganathan Chandrakasan2, Michael Jordan3, Jennifer Leiding4, Abiola Oladapo5, Priti Pednekar6, Kelly Walkovich7 and John Yee5, 1Baylor College of Medicine, Houston, TX, 2Emory University School of Medicine, Atlanta, GA, 3Division of Bone Marrow Transplantation and Immune Deficiency, Cincinnati Children's Hospital Medical Center; Department of Pediatrics, University of Cincinnati College of Medicine, Cincinnati, OH, 4Division of Allergy and Immunology, Department of Pediatrics, Johns Hopkins University; bluebird bio, Cambridge, MA, 5Sobi, Inc., Waltham, MA, 6PRECISIONheor, Bethesda, MD, 7Division of Pediatric Hematology Oncology, Department of Pediatrics, University of Michigan Medical School, Ann Arbor, MI

    Background/Purpose: Macrophage activating syndrome (MAS) is a rare, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD). MAS (a form…
  • Abstract Number: 0761 • ACR Convergence 2023

    EULAR / PreS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis (sJIA) and Adult Onset Still’s Disease (AOSD)

    Bruno Fautrel1, STEPHANE MITROVIC2, Arianna De Matteis3, Sara Bindoli4, Jordi Anton5, Alexandre Belot6, Claudia Bracaglia3, Tamas Constantin7, Lorenzo Dagna8, Alessandro de Bartolo9, Eugen Feist10, Dirk Foell11, Marco Gattorno12, Sophie Georgin-Lavialle13, Roberto Giacomelli14, Alexei Grom15, Yvan Jamilloux16, Katerina Laskari17, Calin Lazar18, Francesca Minoia19, Peter Nigrovic20, Filipa Oliveira Ramos21, Seza Ozen22, Pierre Quartier Dit Maire23, Piero Ruscitti24, Erdal Sag25, Sinisa Savic26, Marie-Elise Truchetet27, Sebastiaan Vastert28, Tanita Wilhelmer29, Carine Wouters30, Loreto Carmona31 and Fabrizio De Benedetti32, 1Sorbonne Université APHP, Paris, France, 2Pitie Salpetriere Hospital, Sorbonne University, APHP, Paris, France, 3IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Italy, 4Rheumatology Unit, department of Medicine,University of Padova, Padova, Italy, 5Hospital Sant Joan de Déu, Pediatric Rheumatology Department, Universitat de Barcelona, Barcelona, Spain, 6Hospices Civils de Lyon, Collonges au mont d'or, France, 7Semmelweis University, Budapest, Hungary, 8Ospedale San Raffaele, Milano, Italy, 9PReS Patient Research Partner, Milan, Italy, 10Helios Department of Rheumatology and Clinical Immunology, Vogelsang-Gommern, Germany, 11University Hospital Münster, Münster, Germany, 12UOC Reumatologia e Malattie Autoinfiammatorie, Genoa, Italy, 13AP-HP, Tenon hospital, Paris, France, 14Policlinico Universitario Campus Bio-Medico, Roma, Italy, 15Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 16Hospices Civils de Lyon, Lyon, France, 17Athens University Medical School, Athens, Greece, 18University of Medicine and Pharmacy, Cluj-Napoca, Romania, 19Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Italy, 20Boston Children's Hospital, Boston, MA, 21Centro Hospitalar Universitário Lisboa Norte, Lisbon, Portugal, 22Hacettepe University Medical Faculty, Ankara, Turkey, 23Necker Hospital, Paris Cedex 15, France, 24University of L'Aquila, L'Aquila, Italy, 25Department of Pediatric Rheumatology, Hacettepe University, Ankara, Turkey, 26Leeds University, Leeds, United Kingdom, 27Bordeaux University Hospital, Bordeaux, France, 28University Medical Center Utrecht, Utrecht, Netherlands, 29EULAR Patient Research Partner, Bludenz, Austria, 30Leuven University, Leuven, Belgium, 31Instituto de Salud Musculoesquelética (InMusc), Madrid, Spain, 32Bambino Gesu Children's Hospital, Division of Rheumatology, Rome, Italy

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are supposed to be age counterparts of the same disease, but no consensus has…
  • Abstract Number: 1143 • ACR Convergence 2023

    Nationwide Analysis of Adult-Onset Still’s Disease with and Without Hemophagocytic Lymphohistiocytosis

    Faria Sami1, Michael Manansala2, Shilpa Arora1 and Augustine Manadan2, 1John H. Stroger Jr. Hospital of Cook County, Chicago, IL, 2Rush University Medical Center, Chicago, IL

    Background/Purpose: Adult-Onset Still's Disease (AOSD) commonly manifests with fever, arthritis, rash, liver dysfunction, lymphadenopathy, and hematologic abnormalities. AOSD is also associated with Hemophagocytic Lymphohistiocytosis (HLH).…
  • Abstract Number: 1660 • ACR Convergence 2023

    Still’s Disease Patients with High Interferon-stimulated Gene Expression Have Enrichment of Rare, de Novo and Recessive Protein Altering Variants in Innate Immune Pathways

    Mariana Correia Marques1, Zuoming Deng2, Navid Chowdhury2, Elizabeth Schmitz3, Alana Platukus4, Stephen Brooks5, Carol Lake2, Ly-Lan Bergeron2, Michelle Millwood2 and Michael Ombrello6, 1National Institute of Arthritis & Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 2National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, 3National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), St. Louis, MO, 4National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Philadelphia, PA, 5Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skins Diseases, National Institutes of Health, Bethesda, MD, 6National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD

    Background/Purpose: Still’s disease (systemic juvenile idiopathic arthritis in children, adult-onset Still’s disease in adults) is an enigmatic inflammatory condition that affects people of all ages.…
  • Abstract Number: 1932 • ACR Convergence 2023

    The Association Between Initial Ferritin and Complications from Adults Onset Stills Disease and Intensive Care Unit Admission

    Olivia Yang1, Caroline Bui1, Scott Kubomoto1, Napatkamon Ayutyanont1, Kimberly Vickery1 and Kumar Venkat2, 1Riverside Community Hospital, Riverside, CA, 2Retired, Irvine, CA

    Background/Purpose: Adult Onset Still's Disease (AOSD) is an autoinflammatory disease characterized by quotidian fever, arthralgia, and a salmon colored rash. Due to its rarity, there…
  • Abstract Number: 2473 • ACR Convergence 2023

    A Consensus Based Algorithm to Screen for Lung Disease in Children with Systemic Juvenile Idiopathic Arthritis

    Holly Wobma1, Ronny Bachrach2, Joseph Farrell2, Margaret Chang3, Megan Day-Lewis1, Fatma Dedeoglu1, Martha Fishman4, Olha Halyabar1, Claudia Harris1, Daniel Ibanez1, Liyoung Kim1, Timothy Klouda4, Katie Krone4, Pui Lee1, Mindy Lo1, Kyle McBrearty1, Esra Meidan1, Susan Prockop5, Aaida Samad4, Mary Beth Son1, Peter Nigrovic3, Alicia Casey4, Joyce Chang3 and Lauren Henderson3, 1Division of Immunology, Boston Children's Hospital, Boston, MA, 2Community Member, Boston, MA, 3Boston Children's Hospital, Boston, MA, 4Division of Pulmonary Medicine, Boston Children’s Hospital, Boston, MA, 5Division of Hematology-Oncology, Boston Children's Hospital, Boston, MA

    Background/Purpose: Over the last decade, lung disease (LD) has become an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). Children with sJIA-LD may be…
  • Abstract Number: 036 • 2023 Pediatric Rheumatology Symposium

    Predictive Factors of Long-lasting Remission Following Anakinra Withdrawal in Patients with Systemic Juvenile Idiopathic Arthritis After Achievement of Clinical Inactive Disease

    Germana Nardini1, Claudia Bracaglia2, Denise Pires Marafon1, Emanuela Sacco3, Arianna De Matteis1, Ivan Caiello1, Giusi Prencipe1, Fabrizio De Benedetti2 and Manuela Pardeo2, 1Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesu', Roma, Italy, 2Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy, 3Fondazione Casa Sollievo della Sofferenza, Pediatria, San Giovanni Rotondo, Italy

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare autoinflammatory disease of unknown etiology. Several uncontrolled studies showed that early treatment with anakinra is associated…
  • Abstract Number: 097 • 2023 Pediatric Rheumatology Symposium

    Survey of Covid-19 Immunization and Infection in Patients with Systemic Juvenile Idiopathic Arthritis and Adult Onset Still’s Disease

    Mariana Correia Marques1, Paul Subrata2, Carol Lake3, Ly-Lan Bergeron4, Rashmi Sinha5, Luciana Peixoto6, Marinka Twilt7 and Michael Ombrello8, 1Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases/Children`s National Hospital, Bethesda, MD, 2NIAID Collaborative Bioinformatics Resource (NCBR), National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD, 3NIH, GAITHERSBURG, MD, 4NIH/NIAMS, Vienna, VA, 5Systemic JIA Foundation, Cincinnati, OH, 6Systemic JIA Foundation, Cincinnati, 7Alberta Children's Hospital, Calgary, AB, Canada, 8Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, North Bethesda, MD

    Background/Purpose: Immunization is one of the most important tools for the control of the Covid-19 pandemic. The safety and effectiveness of the Covid-19 immunizations have…
  • Abstract Number: 113 • 2023 Pediatric Rheumatology Symposium

    Proportion of Patients with a Polyphasic Disease Course in Systemic-onset Juvenile Idiopathic Arthritis May Be Higher in the Age of Cytokine Inhibitors

    Itay Marmor1, Rotem Semo Oz2, Amir hendel3, Guy Hazan4, Kevin Baszis5, Anthony French5, Cuoghi Edens6, Irit Tirosh7, Yonatan Butbul Aviel8, Liora Harel9 and Gil Amarilyo10, 1Dana-Dwek Children's Hospital, Hod Hasharon, Israel, 2Sheba medical center, Herzelyia, Israel, 3Tel Aviv University, Tel Aviv, Israel, 4Soroka University Medical Center, Be'er Sheva, Israel, 5Washington University School of Medicine, St Louis, MO, 6University of Chicago, Chicago, IL, 7Sheba Medical Center, Savyon, Israel, 8Rambam Medical center, Haifa, Israel, 9Scheiders Children Medical Center of Israel, Petah-Tiqva, Israel, 10Schneider Children's Medical Center of Israel, Petach Tikva, Israel

    Background/Purpose: Systemic-onset juvenile idiopathic arthritis (sJIA) is a pediatric autoinflammatory condition, known for significant variability between patients in its severity and long-term outcomes. The classification…
  • Abstract Number: L12 • ACR Convergence 2022

    First Line Treatment Using Recombinant IL-1Receptor Antagonist in New Onset Systemic Juvenile Idiopathic Arthritis Is an Effective Treatment Strategy, Irrespective of HLA DRB1 Background

    Remco Erkens1, Rashmi Sinha2, Alex Pickering3, Grant Schulert4, Alexei Grom4, Lars van der Veken1, Hanneke van Deutekom1, Jorg Calis1, Jorg van Loosdregt5 and Sebastiaan Vastert1, 1University Medical Center Utrecht, Utrecht, Netherlands, 2Systemic JIA Foundation, Cincinnati, OH, 3Systemic JIA Foundation, San Francisso, CA, 4Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 5University Medical Center Utrecht, Wilhelmina Children's Hospital, Zeist, Netherlands

    Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a severe subtype of JIA. Recently, interstitial lung disease (SJIA-LD) has been reported as a severe complication of…
  • Abstract Number: 0872 • ACR Convergence 2022

    Incidence, Risk Factors, and Outcomes of Eosinophilia on IL-1 and IL-6 Inhibitors in Systemic and Non-Systemic Juvenile Idiopathic Arthritis

    Holly Wobma1, Maria Taylor2, Ki Pui Lam1, Helene Brown1, Siobhan Case1, Mia Chandler1, Margaret Chang1, Ezra Cohen1, Megan Day-Lewis1, Olha Halyabar1, Jonathan Hausmann1, Melissa Hazen3, Erin Janssen1, Pui Lee1, Mindy Lo1, Esra Meidan1, Jordan Roberts1, Mary Beth F. Son1, Robert Sundel1, Fatma Dedeoglu1, Peter Nigrovic1, Alicia Casey4, Joyce Chang1 and lauren henderson1, 1Division of Immunology, Boston Children's Hospital, Boston, MA, 2Division of Immunology, Boston Children's Hospital, Brighton, MA, 3Boston Children's Hospital, Boston, MA, 4Division of Pulmonary Medicine, Boston Children's Hospital, Boston, MA

    Background/Purpose: Children with systemic juvenile idiopathic arthritis (sJIA) exposed to IL1/6 inhibitors may develop eosinophilia with an atypical rash, reportedly in association with HLA-DRB1*15:XX. There…
  • Abstract Number: 0562 • ACR Convergence 2022

    Large-Scale Targeted Sequencing Study Links Systemic Juvenile Idiopathic Arthritis with Rare Variants of MEFV, LYST, STXBP2, UNC13D

    Mariana Correia Marques1, Danielle Rubin2, Emily Shuldiner2, Elizabeth Schmitz2, Elizabeth Baskin2, Andrew Patt3, Alexei Grom4, Dirk Foell5, Marco Gattorno6, John Bohnsack7, Rae Yeung8, Sampath Prahalad9, Elizabeth Mellins10, Jordi Antón11, Claudio Len12, Sheila Oliveira13, Patricia Woo14, Seza Ozen15, INCHARGE Consortium16 and Michael Ombrello17, 1National Institute of Arthritis and Musculoskeletal and Skin Diseases / Children`s National Hospital, Bethesda, MD, 2National Institute of Arthritis and Musculoskeletal and Skin Diseases, Bethesda, MD, 3National Center for Advancing Translational Sciences, Bethesda, MD, 4Divisions of Rheumatology, Cincinnati Children’s Hospital Medical Center, Cincinnati, OH, 5University Hospital Münster, Münster, Germany, 6Pediatric Clinic and Rheumatology Unit, IRCCS Istituto Giannina Gaslini, Genoa, Italy, 7University of Utah, Salt Lake City, UT, 8The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, 9Emory + Children's Pediatric Institute, Atlanta, GA, 10Stanford University, Stanford, CA, 11Pediatric Rheumatology Department. Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat, Spain, 12Universidade Federal de São Paulo, São Paulo, Brazil, 13Universidade Federal do Rio de Janeiro, Rio De Janeiro, Brazil, 14University College London, London, United Kingdom, 15Hacettepe University Faculty of Medicine, Ankara, Turkey, 16International Childhood Arthritis Genetics Consortium, Bethesda, MD, 17National Institutes of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD

    Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a genetically complex inflammatory condition. It can be marked by severe systemic inflammation that resembles the hereditary periodic…
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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.

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