Abstracts tagged "Still’s disease"

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: 1929 • ACR Convergence 2025
Economic Burden of Macrophage Activation Syndrome (MAS) in Patients with Still’s Disease (Systemic Juvenile Idiopathic Arthritis (sJIA) and Adult-onset Still’s Disease (AOSD)): Analysis of a US National Administrative Claims Database
Michael Marrone¹, Christopher McPherson² and Abiola Oladapo³, ¹Sobi Inc., Mt Pleasant, SC, ²Sobi, University City, MO, ³Sobi INC, Waltham, MA
Background/Purpose: Macrophage activation syndrome (MAS) is a severe, life-threatening complication of rheumatologic disease. MAS is a form of secondary hemophagocytic lymphohistiocytosis which occurs most frequently…
Abstract Number: 0163 • ACR Convergence 2025
Trends in hospitalization and outcomes among patients admitted with Adult-onset Still’s disease in the United States: INSIGHTS FROM THE NATIONAL INPATIENT SAMPLE (2016-2022)
Mohammed Akram¹, Muhammad Sohaib Asghar², Woo Joo Lee², Basmah Fayaz Yousuf³, Carmen Tormo Carillo⁴ and Savier Aleman Rama⁴, ¹HCA Aventura, Aventura, FL, ²AdventHealth Sebring, Sebring, FL, ³SUNY Downstate Health Sciences University, New York, NY, ⁴HCA Florida Aventura Hospital, Aventura, FL
Background/Purpose: Adult-onset Still's Disease is a rare systemic autoinflammatory disorder characterized by spiking fever, arthralgia/arthritis, skin rash, and elevated inflammatory markers (ferritin). Estimated incidence ranges…
Abstract Number: 1901 • ACR Convergence 2025
Epidemiology of Adult-Onset Still’s Disease in South Korea: A Nationwide Study Using Health Insurance Claims Data
Mihye Kwon¹, Younghee Jung², Chung-il Joung¹ and Jeeyoung Hong¹, ¹Konyang University Hospital, Daejeon, Republic of Korea, ²Chungnam National University Hospital, Daejeon, Republic of Korea
Background/Purpose: Adult-onset Still’s disease (AOSD) is a rare systemic autoinflammatory disorder characterised by heterogeneity in clinical course and long-term outcomes. Despite its rarity being recognised…
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…

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