Abstracts tagged "macrophage activation syndrome"

Abstract Number: 0039 • ACR Convergence 2025
Hemophagocytic Lymphohistiocytosis Gene Variants in Severe COVID-19 Cytokine Storm Syndrome
randy Cron¹, Abhishek Kamath¹, Mingce Zhang¹, Devin Abhser², Lesley Jackson¹ and Walter Winn Chatham³, ¹University of Alabama at Birmingham, Birmingham, AL, ²Kaiser Permanente Research Bank, Oakland, CA, ³University of Nevada, Las Vegas, Las Vegas, NV
Background/Purpose: Severe COVID-19 infection resulting in hospitalization shares features with frequently fatal cytokine storm syndromes (CSS), such as hemophagocytic lymphohistiocytosis (HLH) and 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: 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…
Abstract Number: 0829 • ACR Convergence 2024
Epstein-Barr Virus Infection Triggers Hyperinflammation and Cytokine Storm in Healthy Children
Kailey Brodeur¹, Meng Liu², Rachel Weng², Evan Hsu², Lauren Henderson³, Fatma Dedeoglu², Jane Newburger³, Peter Nigrovic⁴, Mary Beth Son⁴ and Pui Lee⁵, ¹Boston Children's Hospital, Cumberland, RI, ²Boston Children's Hospital, Boston, MA, ³Boston Children's Hospital, Watertown, MA, ⁴Boston Children's Hospital, Brookline, MA, ⁵Boston Children's Hospital, Newton, MA
Background/Purpose: Epstein-Barr virus (EBV) is common pathogen responsible for infectious mononucleosis but also triggers hemophagocytic lymphohistiocytosis (HLH). This variation in the immune response to EBV…
Abstract Number: 0834 • ACR Convergence 2024
Evolution of Lung Inflammation in a Mouse Model of Chronic/Recurrent Macrophage Activation Syndrome
Richard Chhaing, Natsumi Inoue, Jana Latayan and grant schulert, Cincinnati Children's Hospital Medical Center, Cincinnati, OH
Background/Purpose: Systemic juvenile idiopathic arthritis-associated lung disease (SJIA-LD) is a life-threatening complication and associated in >80% of cases with macrophage activation syndrome (MAS); however, the…
Abstract Number: 1765 • ACR Convergence 2024
CD8+ T Cells and Monocytes from Children with Macrophage Activation Syndrome Demonstrate Specific Transcriptional Changes Consistent with T Cell Activation and Expansion of Monocytes Shaped by Interferon and TLR Signaling
Susan Canny¹, Hannah DeBerg², Griffin Gessay², Ailing Lu³, Mary Eckert⁴, Andrea La Bella⁵, Hayley Waterman², Danish Nadeem², Susan Shenoi⁶, Joyce Hui-Yuen⁷, Daniel Campbell², Betsy Barnes⁸ and Jessica Hamerman², ¹University of Washington, Seattle, WA, ²Benaroya Research Institute, Seattle, WA, ³Northwell Health, Manhasset, NY, ⁴Seattle Children's Hospital, Seattle, WA, ⁵Cohen Children's Medical Center, Queens, NY, ⁶Seattle Children's Hospital and Research Center, Mercer Island, WA, WA, ⁷North Shore LIJ Health System, Great Neck, NY, ⁸Feinstein Institutes for Medical Science, Manhasset, NY
Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (sHLH), is a potentially fatal complication of rheumatic diseases. MAS is characterized by a…
Abstract Number: 1768 • ACR Convergence 2024
A Potential Role of Longstanding IL-18 Stimulation in the Susceptibility for Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Greta Rogani¹, Remco Erkens¹, Marein Putmans¹, Rianne Scholman¹, Jorg van Loosdregt² and Sebastiaan Vastert¹, ¹University Medical Center Utrecht, Utrecht, Netherlands, ²University Medical Center Utrecht, La Jolla, CA
Background/Purpose: Macrophage Activation Syndrome (MAS) is a pathologic condition of immune hyperactivation, which occurs in 10-30% of cases of Still’s Disease (SD), the spectrum of…
Abstract Number: 1772 • ACR Convergence 2024
Modeling and Predicting HLH Through Measurement of Immune Synapse Duration, Cytokine Production, and Target Cell Death
Anastasia Frank-Kamenetskii¹, Jemy Varghese², Jeremy Morrissette³, Hannah Klinghoffer⁴, Caroline Diorio⁵, Janis Burkhardt⁶ and Scott Canna², ¹CHOP/UPENN, Philadelphia, PA, ²Children's Hospital of Philadelphia, Philadelphia, PA, ³University of Pennsylvania - Perelman School of Medicine, Philadelphia, PA, ⁴The Children's Hospital of Philadelphia, Philadelphia, PA, ⁵Division of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA, ⁶The Children's Hospital of Philadelphia, Philadelphia
Background/Purpose: Hyperinflammation is a life-threatening systemic inflammatory state most commonly associated with Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activation Syndrome (MAS), but observed in nearly all inflammatory…
Abstract Number: 1782 • ACR Convergence 2024
DOCK2 Mutations and Hyper-Inflammatory Syndromes
Randy Cron, Mingce Zhang and Prescott Atkinson, University of Alabama at Birmingham, Birmingham, AL
Background/Purpose: Cytokine storm syndromes (CSS) are frequently fatal hyper-inflammatory complications of a variety of oncologic, rheumatic, and infectious diseases. Many patients with CSS possess heterozygous…
Abstract Number: 1997 • ACR Convergence 2024
Features of interleukin-1 and interleukin-6 Inhibitor (IL-1i/IL-6i) Related Severe Delayed Adverse Reactions in Subjects with or Without Reaction-risk Associated HLA-DRB1*15
Vivian E Saper¹, Kazutoyo Osoegawa¹, Ruud Verstegen², Marcelo A Fernandez Vina¹ and Lu Tian¹, and Drug Hypersensitivity Consortium, ¹Stanford University, Stanford, CA, ²The Hospital for Sick Children, Toronto, ON, Canada
Background/Purpose: A very serious drug-related adverse event is reported with inhibitors of IL-1 and IL-6 (IL-1i/IL6i). This reaction scores as drug reaction with eosinophilia and…
Abstract Number: 2174 • ACR Convergence 2024
Current Treatment of Macrophage Activation Syndrome Worldwide: The METAPHOR Project, a PReS/PRINTO Real-life International Survey
Francesca Minoia¹, Francesco Baldo², Remco Erkens³, Greta Rogani³, Claudia Bracaglia⁴, Dirk Foell⁵, Marco Gattorno⁶, Marija Jelusic⁷, Jordi Anton⁸, Paul Brogan⁹, Scott Canna¹⁰, Shanmuganathan Chandrakasan¹¹, Randy Cron¹², Fabrizio De Benedetti⁴, Alexei Grom¹³, Merav Heshin Bekenstein¹⁴, AnnaCarin Horne¹⁵, Raju Khubchandani¹⁶, Mao Mizuta¹⁷, Seza Ozen¹⁸, PIERRE QUARTIER¹⁹, Angelo Ravelli⁶, Masaki Shimizu²⁰, grant schulert¹³, Christiaan Scott²¹, Rashmi Sinha²², Nicolino Ruperto²³, Joost Swart³ and Sebastiaan Vastert³, and the PReS MAS/sJIA Working Party and Pediatric Rheumatology International Trial Organization., ¹Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milano, Milan, Italy, ²Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy, ³University Medical Center Utrecht, Utrecht, Netherlands, ⁴IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy, ⁵University Hospital Muenster, Muenster, Germany, ⁶IRCCS Giannina Gaslini, Genoa, Italy, ⁷University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Zagreb, Croatia, ⁸Hospital Sant Joan de Déu. Universitat de Barcelona, Esplugues de Llobregat (Barcelona), Spain, ⁹UCL Institute of Child Health and Great Ormond Street Hospital NHS Foundation Trust, London, United Kingdom, ¹⁰Children's Hospital of Philadelphia, Philadelphia, PA, ¹¹Aflac Cancer and Blood Disorders Center Children's Healthcare of Atlanta Emory University School of Medicine, Atlanta, GA, ¹²University of Alabama at Birmingham, Birmingham, AL, ¹³Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ¹⁴Dana Dwek Children's Hospital, Tel Aviv Medical Center, Tel Aviv University, Binyamina, Israel, ¹⁵Karolinska Institute, Sollentuna, Sweden, ¹⁶SRCC Childrens Hospital, Mumbai, India, ¹⁷Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children's Hospital, Kanazawa, Hyogo, Japan, ¹⁸Department of Pediatrics, Hacettepe University, Ankara, Turkey, ¹⁹Université Paris-Cite, IMAGINE Institute, Necker Children's Hospital, Paris Cedex 15, France, ²⁰Tokyo Medical and Dental University, Bunkyo-ku, Tokyo, Japan, ²¹University of Cape Town, Cape Town, South Africa, ²²Systemic JIA Foundation, Cincinnati, OH, ²³IRCCS Giannina Gaslini, Genova, Italy
Background/Purpose: Despite significant improvement in its management, macrophage activation syndrome (MAS) treatment is still not standardized, due to lack of robust evidence and differences in…
Abstract Number: 2569 • ACR Convergence 2024
A Novel Hyperferritinemia Screen to Aid Differentiation of Hyperinflammatory Disorders
Hallie Carol¹, Adam Mayer², Jemy Varghese³, Zachary Martinez⁴, Caroline Diorio⁴, Paul Tsoukas⁵, Kate Kernan⁶ and Scott Canna⁷, ¹Children's Hospital of Philadelphia, Philadelphia, ²University of Pennsylvania/Children's Hospital of Philadelphia, Philadelphia, PA, ³Division of Pediatric Rheumatology, The Children’s Hospital of Philadelphia, Philadelphia, PA, ⁴Division of Pediatric Oncology, The Children’s Hospital of Philadelphia, Philadelphia, PA, ⁵Hospital for Sick Children/University of Toronto, Toronto, ON, Canada, ⁶Department of Critical Care Medicine, University of Pittsburgh School of Medicine, UPMC Children’s Hospital of Pittsburgh, Pittsburgh, PA, ⁷Children's Hospital of Philadelphia, Philadelphia, PA
Background/Purpose: High ferritin is an important and sensitive biomarker for the diverse and deadly group of cytokine storm syndromes grouped together under the term hemophagocytic…
Abstract Number: 0389 • ACR Convergence 2024
Histopathological Features of Liver Tissue Biopsies in SJIA Patients with and Without Clinical Macrophage Activation Syndrome
Esraa Eloseily¹, Lara Berklite², Jennifer Picarsic¹, grant schulert¹, Rachel Sheridan¹ and Alexei Grom¹, ¹Cincinnati Children's Hospital Medical Center, Cincinnati, OH, ²Cincinnati Children's Hospital Medical Center, Cincinnti, OH
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) can present with or without Macrophage Activation Syndrome (MAS), a severe, potentially life-threatening complication. Liver tissue injury is commonly…

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