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Abstracts tagged "Still’s disease"

  • 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: 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: 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…
  • 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: 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,…
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