Abstracts tagged "macrophage activation syndrome"

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: 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: 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: 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: 1825 • ACR Convergence 2025
Serum proteomic and single cell RNA sequencing analysis reveals macrophage activation syndrome physiology and widespread complement dysregulation are associated with severe dengue infection in a pediatric cohort
Sarah McCuaig¹, Molly Gallagher¹, Yentli E. Soto Albrecht², Francia Acosta³, Shanti Turbi-Cornielle⁴, Reyna Soriano⁴, Eugenia Gonzalez-Diaz⁴, Vincenta Sanchez⁵, Elsa Herrera⁶, Aide Cornielle⁴, Anabel Fernandez¹, Adriana LaMonte¹, Sarah E Henrickson¹, Laura Vella¹, Andrew P Steenhoff⁷ and Edward Behrens⁸, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, ³Ninos Primeros en Salud Program, Consuelo, Dominican Republic, ⁴Universidad Central del Este, San Pedro de Macorís, Dominican Republic, ⁵Robert Reid Cabral Children's Hospital, Santo Domingo, Dominican Republic, ⁶Dr. Antonio Musa Regional Hospital, San Pedro de Macorís, Dominican Republic, ⁷Children's Hospital of Philadelphia, Philadelphia, ⁸CHOP, West Chester, PA
Background/Purpose: Dengue virus (DENV) is a global public health threat that is increasing in incidence and endogenous DENV infections are expected to dramatically rise in…
Abstract Number: 1820 • ACR Convergence 2025
Cytokine profiling in refractory systemic juvenile idiopathic arthritis reveals distinct signatures for macrophage activation syndrome and lung disease.
Taskin Sabit¹, Minyi Yu², Joy Baker¹, Sanjeev Dhakal³, Sam Chiang⁴, Scott Canna⁵, Randy Cron⁶, Lauren Henderson⁷, Karen Onel⁸, Mona Riskalla⁹, Tiphanie Vogel¹⁰, Pui Lee¹¹, Grant Schulert² and Alexei Grom², ¹Cincinnati Children's Hospital Medical Center, Division of Rheumatology, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ³Cincinnati Children hospital, Cincinnati, OH, ⁴Cincinnati Children's Hospital Medical Center, Cincinnnati, OH, ⁵Children's Hospital of Philadelphia, Philadelphia, PA, ⁶University of Alabama at Birmingham, Birmingham, AL, ⁷Boston Children's Hospital, Watertown, MA, ⁸HSS, New York, NY, ⁹University of Minnesota Masonic Children's Hospital, Minneapolis, MN, ¹⁰Baylor College of Medicine, Houston, TX, ¹¹Boston Children's Hospital, Newton, MA
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is a severe inflammatory disorder that affects the joints as well as other organ systems, with complications including Macrophage…
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: 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: 1477 • ACR Convergence 2025
Can Large Language Models Support Clinical Decision-Making in Atypical SLE? A Comparative Analysis
Beste Acar¹, Berkay Aktas¹, Oguzhan Omer Kizilkaya¹, Zekayi Kutlubay² and serdal Ugurlu³, ¹Cerrahpasa Faculty of Medicine, Istanbul University-Cerrahpasa, Istanbul, Turkey, ²Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Department of Dermatology, Istanbul, Turkey, Istanbul, Istanbul, Turkey, ³Istanbul University-Cerrahpasa, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey
Background/Purpose: This study evaluates the contributions of large language models (LLMs) in clinical decision-making for atypical presentations of systemic lupus erythematosus (SLE). It focuses on…
Abstract Number: 1471 • ACR Convergence 2025
Prevalence and Clinical Outcomes of Hemophagocytic Lymphohistiocytosis in Systemic Lupus Erythematosus: A Multi-Institutional Cohort Study
Amir Daneshvar¹, Julia Wajsberg¹, Chelsea Guan¹, Keri Ann Pfeil¹, Elleson Harper¹, Lindsay Frumker², Meghan Gump¹ and Omer Pamuk³, ¹University Hospitals Cleveland Medical Center/Case Western Reserve University, Cleveland, OH, ²University hospitals Cleveland medical center, Highland Heights, OH, ³University Hospitals Cleveland Medical Center/ Case Western Reserve University, Cleveland, OH
Background/Purpose: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with multisystem involvement. A rare but life-threatening hyper-inflammatory hematological complication of SLE is hemophagocytic lymphohistiocytosis…
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: 1153 • ACR Convergence 2025
New Disease Classification in the Block? A 5-year Retrospective Review on Clinical Profiles and Mortality Outcomes of Patients Presenting with Hematoinflammatory Syndromes at a University Center in Florida
Cristine Kuzhuppilly Arcilla¹, Gabriel Kirsch², Michael Cargill² and Gurjit Kaeley³, ¹University of Florida Jacksonville, Jacksonville, FL, ²University of Florida - Jacksonville, Jacksonville, FL, ³UF COM-J, Ponte Vedra Beach, FL
Background/Purpose: The novel diagnosis of VEXAS in 2020 led to the proposed possibility of a new category of hematoinflammatory syndromes linking clinically overlapping hematologic and…
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: 2157 • ACR Convergence 2025
Optimizing the Diagnosis and Treatment of Kawasaki Disease-Associated Macrophage Activation Syndrome: A Clinical Cohort and Literature Analysis
Xi Yang¹, Yuanhao Zhao², Zhihan Tang², Haixia Liu² and Xuemei Tang², ¹Department of Rheumatology & Immunology, Children’s Hospital of Chongqing Medical University, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, National Clinical Research Center for Child Health and Disorders (Chongqing), Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Infection and Immunity, Chongqing, China., Chongqing, China (People's Republic), ²Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Child Rare Diseases in Infection and Immunity, Chongqing, China, Chongqing, China (People's Republic)
Background/Purpose: Through a single-center retrospective cohort study and systematic literature review, we aimed to evaluate the applicability of current diagnostic criteria (HLH-2004/2009 and 2016 sJIA-MAS)…
Abstract Number: 0423 • ACR Convergence 2025
Macrophage activation syndrome-associated proteins and enhanced interferon gamma responsiveness characterize the plasma proteome of patients with Multisystem Inflammatory Syndrome in Children (MIS-C) in a pre-treatment replication single center cohort
Sarah McCuaig¹, Cara Toland², Katharine Konvinse³, Emily Yang³, Paul Utz⁴, Laura Vella¹, Audrey R. Odom John¹, Hamid Bassiri¹ and Edward Behrens⁵, ¹Children's Hospital of Philadelphia, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia, ³Stanford University School of Medicine, Palo Alto, CA, ⁴Stanford University, Stanford, CA, ⁵CHOP, West Chester, PA
Background/Purpose: Multisystem inflammatory syndrome in children (MIS-C) is a rare hyperinflammatory syndrome that follows SARS-CoV-2 infection. Prior plasma proteomic analysis from a 2020 cohort of…

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