Abstracts tagged "Still’s disease"

Abstract Number: 1819 • ACR Convergence 2025
Spatial Transcriptomic Profiling Reveals Interferon Activation and CD8⁺ T Cell Dominance in Systemic Juvenile Idiopathic Arthritis-Macrophage Activation Syndrome Liver Inflammation
Esraa Eloseily¹, Taskin Sabit², Lara Berklite³, Grant Schulert⁴ and Alexei Grom⁴, ¹UT Southwestern Children's Medical Center, Dallas, TX, ²Cincinnati Children's Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ³Cincinnati Children's Hospital, Cincinnati, OH, ⁴Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) with macrophage activation syndrome (MAS) involves severe systemic inflammation and hepatocellular injury. Our prior histopathology studies showed increased CD8⁺…
Abstract Number: 1811 • ACR Convergence 2025
Pharmacogenomic analysis of prediction of IL-1 Inhibitor treatment response in the CARRA First-line Options for Systemic Juvenile Idiopathic Arthritis Treatment (FROST) Study
Mariana Correia Marques¹, Michael Matt², Sophia Chou³, Peter Burbelo⁴, Zuoming Deng⁵, George Tomlinson⁶, Yukiko Kimura⁷, Grant Schulert² and Michael Ombrello⁸, ¹Translational Genetics and Genomics Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), NIH, Rockville, MD, ⁴Adeno-Associated Virus Biology Section, National Institute of Dental and Craniofacial Research (NIDCR), Bethesda, ⁵Biodata Mining and Discovery Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, MD, ⁶University of Toronto, Toronto, ON, Canada, ⁷Hackensack Meridian School of Medicine, New York, NY, ⁸National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), National Institutes of Health (NIH), North Bethesda, MD
Background/Purpose: Interleukin (IL)-1 inhibitors are now regarded as the first line treatment option for systemic juvenile idiopathic arthritis (sJIA), however in up to half of…
Abstract Number: 1671 • ACR Convergence 2025
Emapalumab Treatment for Patients with Differing Presentations of Macrophage Activation Syndrome (MAS) Secondary to Still’s Disease: Results from a Pooled Analysis of Two Prospective Trials
Alexiei GROM¹, Sebastiaan Vastert², Jordi anton³, Pierre Quartier⁴, Bruno Fautrel⁵, Paul Brogan⁶, Edward Behrens⁷, Melissa Elder⁸, Francesca Minoia⁹, Pavla Dolezalova¹⁰, Robert Biesen¹¹, Masaki Shimizu¹², Uwe Ullmann¹³, Adnan Mahmood¹⁴, Andrew Danquah¹³, Elena Burillo¹³, Marco Petrimpol¹³, Steve Mallett¹⁵, Brian Jamieson¹⁶ and Fabrizio De Benedetti¹⁷, ¹Cincinnati Children’s Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, 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, ⁷CHOP, West Chester, PA, ⁸College of Medicine and Division of Pediatric Allergy, Immunology, and Rheumatology, Department of Pediatrics, University of Florida, GAINESVILLE, FL, ⁹Pediatric Immuno-Rheumatology Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy, ¹⁰Paediatric Rheumatology and Autoinflammatory Diseases Unit, General University Hospital, Prague, Czech Republic, ¹¹Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany, Berlin, Germany, ¹²Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan, ¹³Sobi, Basel, Switzerland, ¹⁴Sobi, Stockholm, Sweden, ¹⁵Sobi, Stock, Sweden, ¹⁶Sobi Inc., Morrisville, NC, ¹⁷Bambino Gesu Children's Hospital, Rome, Rome, Italy
Background/Purpose: MAS is a life-threatening complication of Still’s disease characterized by systemic IFNg-driven hyperinflammation. Patients with Still’s disease may present with MAS at any disease…
Abstract Number: 2143 • ACR Convergence 2025
Ruxolitinib for Refractory sJIA/AOSD: A Single Center Case Series
Jacqueline Madison¹, Andrew Grim² and Janna Shim³, ¹University of Michigan, Ann Arbor, MI, ²University of Michigan Medicine, Ann Arbor, MI, ³Midwestern University Chicago College of Osteopathic Medicine, Downers Grove
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) and Adult-onset Still’s disease (AOSD) are rare, chronic, autoinflammatory diseases distinct from other forms of chronic inflammatory arthritis. Potential…
Abstract Number: 1185 • ACR Convergence 2025
Pulmonary Arterial Hypertension in Adults with Still’s Disease: Another Pulmonary Manifestation Associated with HLA-DRB1*15
Stéphane Mitrovic¹, Athénaïs Boucly², Maryvonnick Carmagnat³, Laurent Savale², Xavier Jaïs², Jean-luc Taupin³, Estibaliz Lazaro⁴, Emilie Berthoux⁵, nicolas schleinitz⁶, Maria-Rosa Ghigna⁷, kedra joanna¹, Xavier Mariette⁸, Céline Roussin⁹, Pierre Antoine Juge¹⁰, Marc Humbert², Olivier Sitbon², David Montani² and Bruno Fautrel¹¹, ¹Pitié-Salpêtrière Hospital, Rheumatology Department, APHP, Sorbonne University,, Paris, France, ²Kremlin Bicêtre Hospital, Pulmonology Department, Paris Saclay University, APHP, Le Kremlin Bicêtre, France, ³Saint Louis Hospital, Immunology laboratory, APHP, Paris, France, ⁴Internal Medicine Department, CHU Bordeaux, Bordeaux, France, ⁵Saint Joseph Sain Luc Hospital, Internal Medicine Department, Lyon, Lyon, France, ⁶Aix Marseille university, AP-HM, Marseille, France, ⁷Marie Lannelongue Hospital, Anatomopathology department, Le Plessis-Robinson, France, ⁸Université Paris-Saclay, Le Kremlin Bicetre, France, ⁹Centre Hospitalier Ouest Réunion, Saint Paul, France, ¹⁰Hopital Bichat, APHP, Paris, France, ¹¹Sorbonne Université - APHP, Department of Rheumatology, Hôpital Pitié-Salpêtrière, Inserm UMRS ¹¹³⁶-⁵, PARIS, France, Paris, France
Background/Purpose: Inflammatory lung disease (ILD) in Still’s disease (SD) has recently been described. Pulmonary arterial hypertension (PAH), a rare subtype of pulmonary hypertension (PH), is…
Abstract Number: 2141 • ACR Convergence 2025
Treatment of Refractory Still’s/Systemic Juvenile Idiopathic Arthritis Lung Disease with the bi-specific IL-1Beta/IL-18 neutralizing antibody MAS825
Hallie Carol¹, Monica Manglani², Abass Noor², Edward Behrens³, Jon Burnham⁴, Jessica Lee², Jordan Moreno², Alexandra Chop⁵, Kader Cetin Gedik⁶, Catherine Poholek⁷, Ginger Janow⁸, Lindsay Waqar-Cowles¹ and Scott Canna¹, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia, ³CHOP, West Chester, PA, ⁴Children's Hospital of Philadelphia, Bryn Mawr, PA, ⁵UPMC, Pittsburgh, PA, ⁶UPMC Children's Hospital of Pittsburgh/University of Pittsburgh, Pittsburgh, PA, ⁷University of Pittsburgh, Pittsburgh, PA, ⁸Joseph M. Sanzari Children's Hospital at Hackensack Meridian Health, Hackensack, NJ
Background/Purpose: Despite major advances in the understanding, diagnosis, and treatment of Still’s disease (including Systemic Juvenile Idiopathic Arthritis, sJIA), many patients experience a refractory course.…
Abstract Number: 1179 • ACR Convergence 2025
Efficacy and Safety of Ruxolitinib in Adult Patients With Refractory Connective Tissue Disease-Associated Macrophage Activation Syndrome
Jingjing Li¹, Ran Wang¹, Jie Chen¹, Antao xu¹, Yakai Fu¹, Yanwei Lin¹, Xiaodong Wang¹, Shuang Ye², Fang Du¹ and Qiong Fu³, ¹Department of Rheumatology, Renji Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China, Shanghai, China (People's Republic), ²Renji Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China, Shanghai, Shanghai, China (People's Republic), ³Renji Hospital, Shanghai, Shanghai, China
Background/Purpose: Macrophage activation syndrome (MAS) is a rare and life-threatening complication of connective tissue diseases (CTDs), with approximately 30% of cases being refractory to standard…
Abstract Number: 2139 • ACR Convergence 2025
Real-Life Treatment Strategies for Refractory Still’s Disease: Results from a Worldwide Survey, the METAPHOR Project
Greta Rogani¹, Francesco Baldo², 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 Vastert¹ and Francesca Minoia²⁶, ¹University Medical Center Utrecht, Utrecht, Utrecht, Netherlands, ²ASST-Pini-CTO, Milano, Milan, Italy, 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: The outcome of Still’s disease (SD) has significantly improved due to new therapeutic options [37923864], early biologic initiation [24623686] and treat-to-target strategies [39317417]. Nonetheless,…
Abstract Number: 1159 • ACR Convergence 2025
IL-18 Correlates with Pouchot Score and Inflammatory Biomarkers in Adult-Onset Still’s Disease: Insights From a Repeated Measures Study
Hadeel Abdul-Rehman¹, Karen Gambina² and Bella Mehta³, ¹Hospital for Special Surgery, New York, NY, ²Columbia University Medical Center, Islip, NY, ³Hospital for Special Surgery, Weill Cornell Medicine, Jersey City, NJ
Background/Purpose: Adult-Onset Still’s Disease (AOSD) is a rare systemic inflammatory disease. Interleukin-18 (IL-18) levels are significantly elevated in AOSD patients¹ and may be a promising…
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,…

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