Abstracts tagged "Still’s disease"

Abstract Number: 2138 • ACR Convergence 2025
Factors driving therapeutic decision-making in Still’s Disease: When to Start and When to Stop? Data from the METAPHOR Project Worldwide Survey
Francesco Baldo¹, Greta Rogani², Claudia Bracaglia³, Dirk Foell⁴, Marco Gattorno⁵, Marija Jelusic⁶, Jordi Anton⁷, Paul Brogan⁸, Scott Canna⁹, Randy Cron¹⁰, Fabrizio De Benedetti¹¹, Alexei Grom¹², Merav Heshin Bekenstein¹³, AnnaCarin Horne¹⁴, Raju Khubchandani¹⁵, Mao Mizuta¹⁶, Seza Özen¹⁷, Pierre Quartier Dit Maire¹⁸, Angelo Ravelli¹⁹, Masaki Shimizu²⁰, Grant Schulert¹², Christiaan Scott²¹, Rashmi Sinha²², Nicolino Ruperto²³, Joost Swart²⁴, Bruno Fautrel²⁵, Sebastiaan Vastert² and Francesca Minoia²⁶, ¹ASST-Pini-CTO, Milano, Milan, Italy, ²University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ³IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, ⁴University Hospital Muenster, Muenster, Germany, ⁵IRCCS G. Gaslini, Genova, Genoa, Italy, ⁶University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ⁷Hospital Sant Joan de Düu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁸UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁹Children's Hospital of Philadelphia, Philadelphia, PA, ¹⁰University of Alabama at Birmingham, Birmingham, AL, ¹¹Bambino Gesu Children's Hospital, Rome, Rome, Italy, ¹²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹³Tel Aviv Medical Center Israel, Binyamina, Tel Aviv, Israel, ¹⁴Karolinska University Hospital, Sollentuna, Sweden, ¹⁵SRCC Childrens Hospital Mumbai, Mumbai, India, ¹⁶Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan, Kobe, Japan, ¹⁷Hacettepe University Medical Faculty, Ankara, Turkey, ¹⁸Necker hospital, Paris Cedex ¹⁵, France, ¹⁹IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, ²⁰Tokyo Medical and Dental University, Bunkyo-ku, Kanazawa, Japan, ²¹Childrens Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada, ²²Systemic JIA Foundation, Cincinnati, OH, ²³Université Milano Bicocca and Fondazione IRCSS S. Gerardo dei Tintori, Monza, Monza and Brianza, Italy, ²⁴Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, ²⁵Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France, ²⁶Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy
Background/Purpose: Despite continuous improvement in care and the recent update of international recommendations, relevant discrepancies in the diagnostic and treatment approach to Still’s disease (SD)…
Abstract Number: 0918 • ACR Convergence 2025
Retention of variant forms of TNFR1 in the Golgi induces stress responses and monocyte activation in systemic Juvenile Idiopathic Arthritis (sJIA)
Anthony Cruz¹, Krisztian Csomos¹, Boglarka Ujhazi¹, Hiroto Nakano², Marissa Krantz³, Sophia Chou⁴, Emily Rosenbaum¹, Tianmin Fu⁵, Dirk Foell⁶, Rae Yeung⁷, Pamela Weiss⁸, Faiza Naz⁹, Ly-Lan Bergeron¹, Michelle Millwood¹⁰, Carol Lake¹¹, Emely Verweyen⁶, Grant Schulert¹², Stefania Dell'orso⁹, Hao Wu¹³, Zuoming Deng¹⁴, davide Randazzo¹⁵ and Michael Ombrello¹⁶, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ²NIAMS, NIH, Bethesda, MD, ³University of Rochester, Bethesda, MD, ⁴National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Rockville, MD, ⁵Ohio State University College of Medicine, Columbus, OH, ⁶University Hospital Muenster, Muenster, Germany, ⁷The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁸Childrens Hospital of Philadelphia, Philadelphia, PA, ⁹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), Bethesda, MD, ¹⁰National Institutes of Health (NIH), Bethesda, MD, ¹¹NIH, GAITHERSBURG, MD, ¹²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹³Harvard University, Cambridge, MA, ¹⁴Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ¹⁵Light Imaging Section, Office of Science and Technology, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, Bethesda, ¹⁶National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is severe and poorly understood inflammatory condition. Tumor necrosis factor receptor 1 (TNFR1) is activated by TNF and is…
Abstract Number: 2137 • ACR Convergence 2025
Therapeutic Strategies in Newly Diagnosed Still’s Disease: Real-Life Clinicians’ Choices from the METAPHOR Project Worldwide Survey
Francesco Baldo¹, Greta Rogani², Claudia Bracaglia³, Dirk Foell⁴, Marco Gattorno⁵, Jordi anton⁶, Paul Brogan⁷, Scott Canna⁸, randy Cron⁹, Alexiei GROM¹⁰, Merav Heshin Bekenstein¹¹, Raju Khubchandani¹², Seza Özen¹³, Pierre Quartier¹⁴, Angelo Ravelli¹⁵, Grant Schulert¹⁶, Mao Mizuta¹⁷, Joost Swart¹⁸, Rashmi Sinha¹⁹, AnnaCarin Horne²⁰, Fabrizio De Benedetti²¹, Christiaan Scott²², Marija Jelusic²³, Masaki Shimizu²⁴, Bruno Fautrel²⁵, Nicolino Ruperto²⁶, Sebastiaan Vastert² and Francesca Minoia²⁷, ¹ASST-Pini-CTO, Milano, Milan, Italy, ²University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ³IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, ⁴Universisty Hospital of Muenster, Muenster, ⁵IRCCS G. Gaslini, Genova, Genoa, Italy, ⁶Hospital Sant Joan de Düu. Universitat de Barcelona, Barcelona, Spain, ⁷UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ⁸Children's Hospital of Philadelphia, Philadelphia, PA, ⁹University of Alabama at Birmingham, Birmingham, AL, ¹⁰Cincinnati Children’s Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ¹¹Tel Aviv Medical Center Israel, Binyamina, Tel Aviv, Israel, ¹²Jaslok Hospital and Research Center, Mumbai, India, ¹³Hacettepe University Medical Faculty, Ankara, Turkey, ¹⁴Hôpital Necker-Enfants Malades, Paris, France, ¹⁵IRCCS Istituto Giannina Gaslini, Genoa, Italy, Genoa, Genoa, Italy, ¹⁶Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹⁷Hyogo Prefectural Kobe Children's Hospital, Kobe, Japan, Kobe, Japan, ¹⁸Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, ¹⁹Systemic JIA Foundation, Cincinnati, OH, ²⁰Karolinska University Hospital, Sollentuna, Sweden, ²¹Bambino Gesu Children's Hospital, Rome, Rome, Italy, ²²Childrens Hospital of Eastern Ontario (CHEO), Ottawa, ON, Canada, ²³University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ²⁴Tokyo Medical and Dental University, Bunkyo-ku, Kanazawa, Japan, ²⁵Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France, ²⁶Université Milano Bicocca and Fondazione IRCSS S. Gerardo dei Tintori, Monza, Monza and Brianza, Italy, ²⁷Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy
Background/Purpose: Despite continuous advances in care and the recent publication of updated international recommendations, relevant discrepancies in the management of Still’s disease (SD) may still…
Abstract Number: 0821 • ACR Convergence 2025
Flipping The Switch – Classical Complement Activation closely linked to IFN-signalling in Stills Disease
Freya Huijsmans¹, Alejandra Bodelón de Frutos¹, Lyanne Sijbers¹, Susanne Benseler², Joost Swart³, Rae Yeung⁴, Sebastiaan Vastert⁵ and Jorg van Loosdregt¹, ¹Pediatric Rheumatology & Immunology, University Medical Center Utrecht, Wilhelmina Children's Hospital, Utrecht, Utrecht, Netherlands, ²Cumming School of Medicine, Department of Pediatrics, University of Calgary, Calgary, AB, Canada, ³Wilhelmina Children's Hospital / UMC Utrecht, Utrecht, Utrecht, Netherlands, ⁴The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada, ⁵University Medical Center Utrecht, Utrecht, Utrecht, Netherlands
Background/Purpose: Stills disease (SD) is an autoinflammatory syndrome characterized by severe innate immune dysregulation. The complement system, an essential component of innate immunity, can drive…
Abstract Number: 2135 • ACR Convergence 2025
Medication Use and Disease Activity in Systemic Juvenile Idiopathic Arthritis in the Childhood Arthritis and Rheumatology Research Alliance Registry
Christina Gulla¹, Mary Beth Son², Tara Lozy³, Yukiko Kimura⁴ and Ginger Janow⁵, ¹Hackensack University Medical Center, Hackensack, NJ, ²Boston Children's Hospital, Boston, MA, ³Joseph M. Sanzari Children's Hospital, Center for Discovery and Innovation, Hackensack Meridian School of Medicine, Nutley, ⁴Hackensack Meridian School of Medicine, New York, NY, ⁵Joseph M. Sanzari Children's Hospital at Hackensack Meridian Health, Hackensack, NJ
Background/Purpose: Historically, treatment for systemic juvenile idiopathic arthritis (sJIA) included high dose glucocorticoids (GC) and conventional systemic (cs) disease modifying anti-rheumatic drugs (DMARDs) with significant…
Abstract Number: 0782 • ACR Convergence 2025
Expansion of T-bet⁺ Age-Associated B Cells Is Associated with Clinical Complications in Still’s Disease
Krisztian Csomos¹, Boglarka Ujhazi¹, Mariana Correia Marques², Emily Rosenbaum¹ and Michael Ombrello³, ¹National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Bethesda, MD, ²Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Still’s disease is a rare and severe autoinflammatory disorder characterized by daily spiking fevers, arthritis, an evanescent rash, and prominent systemic features, including lymphadenopathy,…
Abstract Number: 2134 • ACR Convergence 2025
Krebs von den Lungen-6 (KL-6) as a Potential Diagnostic Biomarker of Lung Disease in Pediatric Systemic Juvenile Idiopathic Arthritis: Preliminary Findings from a Multisite US Cohort
Eileen Rife¹, Lexi Auld², Guihua Zhai³, Esraa Eloseily⁴, Grant Schulert⁵ and Yukiko Kimura⁶, ¹University of Alabama at Birmingham, birmingham, AL, ²Cincinnati Children's Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ³UAB, Birmingham, ⁴UT Southwestern Children's Medical Center, Dallas, TX, ⁵Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁶Hackensack Meridian School of Medicine, New York, NY
Background/Purpose: Children with Systemic Juvenile Idiopathic Arthritis (SJIA) who develop lung disease (LD) are at significantly increased risk of serious complications and even death. Early…
Abstract Number: 0781 • ACR Convergence 2025
Comprehensive mass cytometry analyses of disease-related cells in adult-onset Still’s disease
Hiroto Yoshida¹, Mayu Magi¹, Hiroya Tamai², Kotaro Matsumoto², Keiko Yoshimoto², Tetsuhiro Soeda¹ and Yuko Kaneko², ¹Chugai Pharmaceutical Co., Ltd. Product Research Dept., Yokohama, Kanagawa, Japan, ²Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, Shinjuku-ku, Tokyo, Japan
Background/Purpose: Adult-onset Still's Disease (AOSD) is a rare autoinflammatory disorder characterized by high fever, rash, and arthritis. Although overactivation of macrophages, an increase in specific…
Abstract Number: 2133 • ACR Convergence 2025
Examination of HLA-DRB1*15-linked Candidate Antigens in Still’s Disease with and without Lung Disease and Features of Drug Hypersensitivity
Dale Kobrin¹, Garrett Brown², Mariana Correia Marques¹, Carol Lake³, Michelle Millwood⁴, Lisa Workman⁵, Monica Lawrence⁵, Zuoming Deng⁶, Sanchita Das² and Michael Ombrello⁷, ¹Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²Department of Laboratory Medicine, National Institutes of Health, Bethesda, MD, ³NIH, GAITHERSBURG, MD, ⁴National Institutes of Health (NIH), Bethesda, MD, ⁵Division of Asthma, Allergy, and Immunology, University of Virginia School of Medicine, Charlottesville, VA, ⁶Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁷National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Lung disease in systemic juvenile idiopathic arthritis and adult-onset Still’s disease (Still’s-LD) is a severe manifestation that strongly associates with features of drug hypersensitivity…
Abstract Number: 0780 • ACR Convergence 2025
Baseline Pharmacodynamic Markers and Response to Emapalumab in Children and Adults with Macrophage Activation Syndrome (MAS) in Still’s Disease: Results from a Pooled Analysis of Two Prospective Trials
Edward Behrens¹, Sebastiaan Vastert², Jordi anton³, Pierre Quartier⁴, Bruno Fautrel⁵, Paul Brogan⁶, Melissa Elder⁷, Francesca Minoia⁸, Pavla Dolezalova⁹, Robert Biesen¹⁰, Masaki Shimizu¹¹, Uwe Ullmann¹², Adnan Mahmood¹³, Andrew Danquah¹², Elena Burillo¹², Marco Petrimpol¹², Steve Mallett¹⁴, Brian Jamieson¹⁵, Alexiei GROM¹⁶ and Fabrizio De Benedetti¹⁷, ¹CHOP, West Chester, PA, ²University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ³Hospital Sant Joan de Düu. Universitat de Barcelona, Barcelona, Spain, ⁴Hôpital Necker-Enfants Malades, Paris, France, ⁵Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France, ⁶Great Ormond Street Hospital for Children NHS Foundation Trust and University College London Great Ormond Street Institute of Child Health, London, United Kingdom, ⁷College of Medicine and Division of Pediatric Allergy, Immunology, and Rheumatology, Department of Pediatrics, University of Florida, GAINESVILLE, FL, ⁸Pediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, ⁹Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, ¹⁰Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany, Berlin, Germany, ¹¹Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan, ¹²Sobi, Basel, Switzerland, ¹³Sobi, Stockholm, Sweden, ¹⁴Sobi, Stock, Sweden, ¹⁵Sobi Inc., Morrisville, NC, ¹⁶Cincinnati Children’s Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ¹⁷Bambino Gesu Children's Hospital, Rome, Rome, Italy
Background/Purpose: MAS is a life-threatening complication of Still’s disease, characterized by IFNg-driven macrophage activation and systemic hyperinflammation. Chemokine C-X-C motif ligand 9 (CXCL9) is released…
Abstract Number: 2130 • ACR Convergence 2025
Systemic juvenile idiopathic arthritis- Fifteen-year experience from a tertiary centre at Bristol, United Kingdom
Ashwini Batchu Prithvi, Chaitra Govardhan, Bushra Aladaileh and Athimalaipet V Ramanan, Bristol Royal Hospital for Children, Bristol, United Kingdom
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a chronic disease that results in significant morbidity and mortality in children1. Improved understanding of the pathophysiology of…
Abstract Number: 0778 • ACR Convergence 2025
Clinical significance of non-infectious increased procalcitonin in Still’s disease: A predictor of macrophage activation syndrome
Erdem Bektas¹, Burcu Ceren Uludogan², Büşra Fırlatan Yazgan³, Ozgur Can Kilinc⁴, Beste Acar⁴, Oguzhan Omer Kizilkaya⁴, Aysenur Yilmaz⁵, Busra Yuce⁶, serdal Ugurlu⁷, Umut Kalyoncu³, Timucin Kasifoglu² and Cemal Bes⁸, ¹Istanbul University, Institute of Graduate Studies in Health Sciences, Immunology, Istanbul, Turkey, ²Division of Rheumatology, Department of Internal Medicine, Eskisehir Osmangazi University, Eskisehir, Turkey, ³Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey, Ankara, Turkey, ⁴Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey, ⁵Department of Rheumatology, Basaksehir Cam and Sakura City Hospital, University of Health Sciences, Istanbul, Turkey, ⁶Department of Internal Medicine, Basaksehir Cam and Sakura City Hospital, University of Health Sciences, Istanbul, Turkey, ⁷Istanbul University-Cerrahpasa, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey, ⁸University of Health Sciences, Basaksehir Çam and Sakura City Hospital, Istanbul, Turkey
Background/Purpose: Still's disease (SD) is a autoinflammatory disease (AID) characterized by a wide range of clinical manifestations and can exhibit life-threatening macrophage activation syndrome (MAS).…
Abstract Number: 2124 • ACR Convergence 2025
Reliability and Validation of the Physician’s Global Assessment of Lung Disease (PGALD) in Systemic Juvenile Idiopathic Arthritis -Associated Lung Disease (SJIA-LD)
Eileen Rife¹, Guihua Zhai², Mekibib Altaye³, Jennifer Andringa⁴, Hermine Brunner⁵, Scott Canna⁶, Lauren Henderson⁷, Yukiko Kimura⁸, Scott Lieberman⁹, Mona Riskalla¹⁰, Tiphanie Vogel¹¹, Holly Wobma¹² and Grant Schulert⁵, ¹University of Alabama at Birmingham, birmingham, AL, ²UAB, Birmingham, ³Cincinnati Children's Hospital, Cincinnati, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, ⁵Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ⁶Children's Hospital of Philadelphia, Philadelphia, PA, ⁷Boston Children's Hospital, Watertown, MA, ⁸Hackensack Meridian School of Medicine, New York, NY, ⁹University of Iowa, Iowa City, IA, ¹⁰University of Minnesota Masonic Children's Hospital, Minneapolis, MN, ¹¹Baylor College of Medicine, Houston, TX, ¹²Boston Children's Hospital, Boston, MA
Background/Purpose: The physician global assessment of lung disease (PGALD) is a recently proposed disease activity measure for patients with systemic juvenile idiopathic arthritis-associated lung disease…
Abstract Number: 0416 • ACR Convergence 2025
Outcomes Of Children with Juvenile Idiopathic Arthritis Receiving Biological Disease-Modifying Anti-Rheumatic Drugs: Retrospective Analysis of A Real-World Experience From A Resource-Limited Setting
Narendra Bagri¹, Pavneet Kaur², Farheen KS², Bala Siva rama Krishna J², Banoth Sreesanth², Ayisha KP², Bareddy Sai Thrisha Reddy², ASHISH DATT UPADHYAY², RAKESH LODHA² and Sushil Kumar kabra², ¹All India Institute of Medical Sciences(AIIMS), New Delhi, Delhi, India, ²All India Institute of Medical Sciences, New Delhi, India
Background/Purpose: We aimed to study the outcomes (remission, flare, and adverse events) of biological disease-modifying anti-rheumatic drugs (bDMARD) in children with JIA from a low-middle-income…
Abstract Number: 2022 • ACR Convergence 2025
Clinical Predictors of Macrophage Activation Syndrome and Treatment Outcomes in Adult-Onset Still’s Disease: A 24-Year Single-Center Experience
Oguzhan Omer Kizilkaya¹, Beste Acar¹, Berkay Aktas¹, Ozgur Can Kilinc¹ and serdal Ugurlu², ¹Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey
Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare autoinflammatory disorder with heterogeneous manifestations. Its most severe complication, macrophage activation syndrome (MAS), occurs in up to…

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