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,…