Abstracts tagged "macrophage activation syndrome"

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: 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: 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: 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: 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: 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…
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…

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].