Abstracts tagged "Still’s disease"

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…
Abstract Number: 0358 • ACR Convergence 2025
Different Perspectives between Physicians and Patients on Treatment Priorities and Challenges in Still’s Disease
Gisella Beatrice Beretta¹, Luciana Pereira², Greta Rogani³, Francesco Baldo⁴, Claudia Bracaglia⁵, Dirk Foell⁶, Marco Gattorno⁷, Marija Jelusic⁸, Sebastiaan Vastert³, Rashmi Sinha⁹ and Francesca Minoia¹⁰, ¹Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, Milan, Italy, ²Systemic JIA Foundation, Cincinnati, ³University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ⁴ASST-Pini-CTO, Milano, Milan, Italy, ⁵IRCCS Ospedale Pediatrico Bambino Gesu', Rome, Rome, Italy, ⁶University Hospital Muenster, Muenster, Germany, ⁷IRCCS G. Gaslini, Genova, Genoa, Italy, ⁸University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ⁹Systemic JIA Foundation, Cincinnati, OH, ¹⁰Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy
Background/Purpose: Despite therapeutic advances, major concerns and disparities persist in the care of Still’s Disease (SD). Capturing both patient/caregiver and physician perspectives is essential to…
Abstract Number: L09 • ACR Convergence 2024
Efficacy and Safety of Tofacitinib in Patients with Active Systemic Juvenile Idiopathic Arthritis
Hermine Brunner¹, Caifeng Li², Kogie Chinniah³, Yosef Uziel⁴, Olga Synoverska⁵, Sujata Sawhney⁶, Inmaculada Calvo Penades⁷, Ingrid Clara Louw⁸, Meiping Lu⁹, Pooja Nikunj Patel¹⁰, Pamela F. Weiss¹¹, Cheng Chang¹², Ivana Vranic¹³, Shixue Liu¹⁴, Annette Diehl¹⁵, Jose L. Rivas¹⁶, Carol A. Connell¹⁷, Gary G. Koch¹⁸, Alberto Martini¹⁹, Daniel J. Lovell¹, Nicolino Ruperto²⁰ and the PRINTO and PRCSG investigators, ¹Cincinnati Children’s Hospital Medical Center, and University of Cincinnati, Cincinnati, OH, ²Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health, Bejing, China, ³Department of Paediatrics and Child Health, University of Kwa-Zulu, and Enhancing Care Foundation, Durban, South Africa, ⁴Pedriatric Rheumatology Unit, Department of Pediatrics, Meir Medical Center and Israel Tel Aviv University School of Medicine, Kfar Saba, Israel, ⁵Ivano-Frankivsk National Medical University, Ivano-Frankivsk, Ukraine, ⁶Sir Ganga Ram Hospital, New Delhi, India, ⁷Hospital Universitario y Politécnico La Fe, Valencia, Spain, ⁸Panorama Medical Centre, Cape Town, South Africa, ⁹Children's Hospital, Zhejiang University School of Medicine, National Clinical Research Center for Child Health, Hangzhou, China, ¹⁰Ann & Robert H. Lurie Children’s Hospital of Chicago; Northwestern University School of Medicine, Chicago, IL, ¹¹Division of Rheumatology, Children's Hospital of Philadelphia, and University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, ¹²Pfizer Inc, New York, NY, ¹³Pfizer Ltd, Tadworth, United Kingdom, ¹⁴Pfizer Inc, Shanghai, China, ¹⁵Pfizer Inc, Collegeville, PA, ¹⁶Pfizer SLU, Madrid, Spain, ¹⁷Pfizer Inc, Groton, CT, ¹⁸University of North Carolina at Chapel Hill, Chapel Hill, NC, ¹⁹University of Genoa, Genoa, Italy, ²⁰Università Milano Bicocca, Milano, and IRCCS Fondazione San Gerardo dei Tintori, Monza, Italy
Background/Purpose: Tofacitinib (TOF) has been shown to be efficacious in the treatment of polyarticular course JIA, including systemic JIA (sJIA) without active systemic features. Here…
Abstract Number: L19 • ACR Convergence 2024
Efficacy and Safety of Emapalumab in Children and Adults with Macrophage Activation Syndrome (MAS) in Still’s Disease: Results from a Phase 3 Study and a Pooled Analysis of Two Prospective Trials
Alexei Grom¹, Uwe Ullman², Adnan Mahmood³, Josefin Blomkvist³, Brian Jamieson⁴ and Fabrizio De Benedetti⁵, ¹Cincinnati Children’s Hospital, Division of Rheumatology, Cincinnati, OH, ²Sobi, Basel, Switzerland, ³Sobi, Stockholm, Sweden, ⁴Sobi, Inc., Morrisville, NC, ⁵Bambino Gesu Children's Hospital, Rome, Italy
Background/Purpose: MAS is a life-threatening complication of Still’s disease, characterized by interferon-gamma (IFNg)-driven macrophage activation and systemic hyperinflammation. Emapalumab, an anti-IFNg antibody, binds free and…

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.

Accepted abstracts are made available to the public online in advance of the meeting and are published in a special online supplement of our scientific journal, Arthritis & Rheumatology. Information contained in those abstracts may not be released until the abstracts appear online. In an exception to the media embargo, academic institutions, private organizations, and companies with products whose value may be influenced by information contained in an abstract may issue a press release to coincide with the availability of an ACR abstract on the ACR website. However, the ACR continues to require that information that goes beyond that contained in the abstract (e.g., discussion of the abstract done as part of editorial news coverage) is under media embargo until 10:00 AM CT on October 25. Journalists with access to embargoed information cannot release articles or editorial news coverage before this time. Editorial news coverage is considered original articles/videos developed by employed journalists to report facts, commentary, and subject matter expert quotes in a narrative form using a variety of sources (e.g., research, announcements, press releases, events, etc.).

Violation of this policy may result in the abstract being withdrawn from the meeting and other measures deemed appropriate. Authors are responsible for notifying colleagues, institutions, communications firms, and all other stakeholders related to the development or promotion of the abstract about this policy. If you have questions about the ACR abstract embargo policy, please contact ACR abstracts staff at [email protected].