Abstracts tagged "Still’s disease"

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…
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: 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].