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Abstracts tagged "Juvenile idiopathic arthritis"

  • Abstract Number: 0137 • ACR Convergence 2022

    Polyarticular Arthritis and Osteolysis Caused by Mutations in the ASAH1 Gene: Farber Disease Clinical Presentations in the First-ever Natural History Study

    Alexander Solyom1, Kathleen Crosby2, Nils Confer2 and Jaime Lopez Valdez3, 1Aceragen, Basel, Switzerland, 2Aceragen, Durham, NC, 3Centenario Hospital Miguel Hidalgo, Aguascalientes, Mexico

    Background/Purpose: Farber disease is frequently misdiagnosed as polyarticular juvenile idiopathic arthritis or seronegative rheumatoid arthritis which leads to a delay in diagnosis for many patients.…
  • 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: 1418 • ACR Convergence 2022

    Change in Disease Activity and Occurrence of Adverse Events After Initiation of Etanercept in Pediatric Patients with Juvenile Psoriatic Arthritis in the CARRA Registry

    Colleen Correll1, Scott Stryker2, David Collier3, Anne Dennos4, Stephen Balevic5, Thomas Phillips5 and Tim Beukelman6, 1University of Minnesota, Minneapolis, MN, 2Amgen Inc., San Francisco, CA, 3Amgen Inc., Simi Valley, CA, 4Duke University, Durham, NC, 5Duke Clinical Research Institute, Durham, NC, 6Childhood Arthritis and Rheumatology Research Alliance (CARRA), Birmingham, AL

    Background/Purpose: Juvenile psoriatic arthritis (JPsA) constitutes ~5% of juvenile idiopathic arthritis (JIA). Several therapeutics are available for JPsA; however, given the low JPsA incidence, important…
  • 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…
  • 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: 1679 • ACR Convergence 2022

    The Childhood Arthritis and Rheumatology Research Alliance Start Time Optimization of Biologic Therapy in Polyarticular JIA (STOP-JIA) Study: Three-Year Outcomes

    Yukiko Kimura1, Sarah Ringold2, George Tomlinson3, Laura Schanberg4, Anne Dennos5, MaryEllen Riordan6, Vincent Del Gaizo7, Katherine Murphy8, Pamela Weiss9, Brian Feldman10, Mei Sing Ong11 and Marc Natter12, 1Hackensack Meridian Health, New York, NY, 2Janssen, Seattle, WA, 3University of Toronto, Toronto, ON, Canada, 4Duke University Medical Center, Durham, NC, 5Duke University, Durham, NC, 6Hackensack Meridian Health, Hackensack, NJ, 7CARRA, Inc, Washington, DC, 8CARRA, Inc, New Orleans, LA, 9Children's Hospital of Philadelphia, Glen Mills, PA, 10Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute; Department of Paediatrics, University of Toronto, Toronto, ON, Canada, 11Harvard Pilgrim Institute, Boston, MA, 12Boston Children's Hospital, Boston, MA

    Background/Purpose: The CARRA STOP-JIA study compared the effectiveness of the CARRA Consensus Treatment Plans (CTPs) in achieving clinically inactive disease (CID) in untreated polyarticular JIA…
  • Abstract Number: 0729 • ACR Convergence 2022

    Understanding the Practice and Process of Patient Reported Outcome Measures Collection in North American Pediatric Rheumatology Clinics: A Survey of the Pediatric Rheumatology-Care and Outcomes Improvement Network

    Y. Ingrid Goh1, Esi Morgan2, Meghan Ryan3, Beth Gottlieb4 and Nancy Pan5, 1Division of Rheumatology, The Hospital for Sick Children; Child Health Evaluative Services, SickKids Research Institute, Toronto, ON, Canada, 2Seattle Children's Hospital, Seattle, WA, 3University of Minnesota, Vadnais Heights, MN, 4Cohen Children's Medical Center, Lake Success, NY, 5Hospital for Special Surgery, New York, NY

    Background/Purpose: Patients/proxies (Pts) complete patient reported outcome measures (PROMs) to inform their healthcare team about their health status. PROMs completed by Pts prior to their…
  • 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: 1934 • ACR Convergence 2022

    Transition Readiness Before versus After Adolescents with Rheumatic Disease Transition to Adult Care

    Christina Ma1, Alessana Carmona2, Habeba Talaat2, Julie Herrington3, Tania Cellucci4, Stephanie Garner2, Mark Matsos2, KAREN BEATTIE2 and Michelle Batthish2, 1McMaster University, Canada, ON, Canada, 2McMaster University, Hamilton, ON, Canada, 3ACPAC, Hamilton, ON, Canada, 4McMaster Children's Hospital, Hamilton, ON, Canada

    Background/Purpose: The transition from pediatric to adult rheumatology care is associated with increased disease activity and morbidity for patients with rheumatic disease. Consequently, there has…
  • Abstract Number: L20 • ACR Convergence 2021

    Efficacy of Emapalumab, an Anti-IFNγ Antibody in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA) Who Had Failed High-Dose Glucocorticoids (GCs)

    Fabrizio De Benedetti1, Alexei Grom2, Paul Brogan3, Claudia Bracaglia1, Manuela Pardeo1, Giulia Marucci1, Despina Eleftheriou3, Charalampia Papadopoulou3, Pierre Quartier4, Jordi Antón5, Rikke Frederiksen6, Veronica Asnaghi6 and Cristina De Min6, 1Bambino Gesù Children's Hospital IRCCS, Rome, Italy, 2Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, 3UCL Institute of Child Health, and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, 4Université de Paris, IMAGINE Institute, RAISE reference centre, Pediatric Immuno-Hematology and Rheumatology Unit, Necker Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France, 5Hospital Sant Joan de Déu, Division of Pediatric Rheumatology, University of Barcelona, Barcelona, Spain, 6Swedish Orphan Biovitrum AG (Sobi), Basel, Switzerland

    Background/Purpose: MAS is a life-threatening complication of rheumatic diseases, occurring most frequently in sJIA. The mainstay of MAS treatment is high dose GCs; however, GCs…
  • Abstract Number: 0241 • ACR Convergence 2021

    Psoriasis Rate Is Increased by the Exposure to TNF Inhibition in Children with JIA

    yongdong Zhao1, Erin Sullivan2, Mary Beth Son3 and Timothy Beukelman4, 1University of Washington, Redmond, WA, 2Seattle Children's Research Institute, Seattle, WA, 3Boston Children's Hospital, Brookline, MA, 4University of Alabama at Birmingham, Birmingham, AL

    Background/Purpose: Paradoxical psoriasis has been increasingly reported in adults after exposure to tumor necrosis factor inhibitors (TNFi). Systematic studies in the pediatric population are lacking.…
  • Abstract Number: 0261 • ACR Convergence 2021

    Methotrexate Withdrawal and Outcomes in Participants with Well-controlled Non-systemic JIA Within the CARRA Registry

    Marinka Twilt1, Kevin Baszis2, Sarah Ringold3, Timothy Beukelman4, Anne Dennos5, Peter Shrader6 and Daniel Horton7, 1Alberta Children's Hospital, Calgary, AB, Canada, 2Washington University School of Medicine, St Louis, MO, 3Seattle Children's Hospital, Seattle, WA, 4University of Alabama at Birmingham, Birmingham, AL, 5Duke University, Durham, NC, 6Duke Clinical Research Institute, Durham, NC, 7Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ

    Background/Purpose: Methotrexate (MTX) is currently the recommended first-line conventional DMARD for the treatment of JIA with oligo- or polyarthritis. There are limited data on how…
  • Abstract Number: 1622 • ACR Convergence 2021

    Mind the Gap: The Experience of Adolescents in a Rheumatology Transition Clinic

    Claire Fine1, Karen Beattie1, Tania Cellucci2, Liane Heale1, Mark Matsos1, Stephanie Garner1 and Michelle Batthish1, 1McMaster University, Hamilton, ON, Canada, 2McMaster Children's Hospital, Hamilton, ON, Canada

    Background/Purpose: The transition from pediatric to adult healthcare is a critical time for the wellbeing of patients with chronic illness including rheumatologic disease. Low patient…
  • 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: 0262 • ACR Convergence 2021

    High Degree of Inter-patient Heterogeneity in Synoviocyte Hyperplasia and Immune Cells Infiltration in the Synovium of Juvenile Idiopathic Arthritis Patients

    Clément TRIAILLE1, Cécile BOULANGER2, Tatiana SOKOLOVA1, Laurent MERIC de BELLEFON3, Adrien NZEUSSEU TOUKAP4, Christine GALANT5, Nisha LIMAYE6, Bernard LAUWERYS7 and Patrick DUREZ8, 1Pôle de pathologies rhumatismales systémiques et inflammatoires, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium, 2Service d’Hématologie, Oncologie et Rhumatologie pédiatrique, Cliniques Universitaires Saint-Luc, Brussels, Belgium, 3Service de Rhumatologie, Cliniques Universitaires Saint-Luc, Brussels, Belgium, 4Rheumatology department, Cliniques Universitaires Saint-Luc, Institut de Recherche Expérimentale et Clinique (IREC), Université catholique de Louvain, Brussels, Belgium, 5Service d’Anatomie Pathologique, Cliniques Universitaires Saint-Luc, Brussels, Belgium, 6Genetics of Autoimmune Diseases and Cancer, de Duve Institute, Université catholique de Louvain, Brussels, Belgium, 7UCB Pharma, Brussels, Belgium, 8Pôle de Recherche en Rhumatologie, Institut de Recherche Expérimentale et Clinique, Brussels, Belgium

    Background/Purpose: Increasing evidence indicates that synovial tissue analysis can deliver pathophysiological insights but also individual clinically-relevant information in adult-onset inflammatory arthritides. Little is known about…
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