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
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
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
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…Abstract Number: 2143 • ACR Convergence 2025
Ruxolitinib for Refractory sJIA/AOSD: A Single Center Case Series
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
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
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
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
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
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
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
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
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.
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
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
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…
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