Abstracts tagged "macrophage activation syndrome"

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: 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: 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…
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…

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