Abstract Number: 0363 • ACR Convergence 2023
Allogenic Hematopoietic Stem Cell Transplant for Children with Refractory Systemic Juvenile Idiopathic Arthritis and sJIA-Associated Lung Disease: 6-month Post-Transplant Outcomes from an International Cohort
Background/Purpose: Refractory systemic juvenile idiopathic arthritis (sJIA) in children can be complicated by repeated episodes of macrophage activation syndrome (MAS) or sJIA-related lung disease (sJIA-LD).…Abstract Number: 0378 • ACR Convergence 2023
Characteristics of Macrophage Activation Syndrome in Systemic JIA Patients Receiving Anakinra as First-line Treatment
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a severe inflammatory disease with auto-inflammatory characteristics. The introduction of targeted biologic therapies has revolutionized the treatment and…Abstract Number: 0758 • ACR Convergence 2023
Clinical and Biological Characteristics of Children and Adults Affected with Still’s Disease: A Systematic Review and Meta-analysis Informing the 2023 EULAR/PReS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis and Adult-onset Still’s Disease
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are part of the non-familial (sporadic) systemic inflammatory disorders and are frequently mentioned as…Abstract Number: 0759 • ACR Convergence 2023
Derivation and Validation of Four Patient Clusters in Still’s Disease, Results from GIRRCS AOSD-study Group and AIDA Network Still Disease Registry
Background/Purpose: Still's disease is a rare inflammatory disorder, these patients have a highly heterogeneous disease according to age of onset, clinical presentation, presence of life-threatening…Abstract Number: 0760 • ACR Convergence 2023
Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still’s Disease Treated with Emapalumab: The REAL-HLH Study
Background/Purpose: Macrophage activating syndrome (MAS) is a rare, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD). MAS (a form…Abstract Number: 0803 • ACR Convergence 2023
IL10 Inhibits Toll-like Receptor-9-induced T Cell Receptor-mediated T Cell Activation in Macrophage Activation Syndrome
Background/Purpose: Macrophage activation syndrome (MAS) is a potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA). Gene expression pathway analyses have identified altered Toll-like receptor…Abstract Number: 0865 • ACR Convergence 2023
Reactive Oxygen Species-scavenging Nanoparticles Target Macrophage Polarization for Osteoarthritis Therapy
Background/Purpose: Unbalanced M1/M2 polarization of synovial macrophages plays vital roles in the symptomatic and structural progression of osteoarthritis (OA). The accumulation of intracellular reactive oxygen…Abstract Number: 1662 • ACR Convergence 2023
Chronic Excess IL-18 Induces NK Deficiency, but Drives Hyperinflammation via CD8 T-cell Cytokine Overproduction and Selective Immunodeficiency
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) and Macrophage Activation Syndrome (MAS) are associated with highly elevated peripheral blood levels of the inflammasome-activated cytokine IL-18 and…Abstract Number: 1711 • ACR Convergence 2023
CD14+ Monocytes Demonstrate a Unique Transcriptional Signature in Macrophage Activation Syndrome, Highlighting a Role for Interferons and Identifying Putative Hemophagocytes in Circulation
Background/Purpose: Macrophage activation syndrome (MAS) is a potentially fatal complication of rheumatic diseases. MAS is characterized by a dysfunctional hyperinflammatory response in which there is…Abstract Number: 2473 • ACR Convergence 2023
A Consensus Based Algorithm to Screen for Lung Disease in Children with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Over the last decade, lung disease (LD) has become an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). Children with sJIA-LD may be…Abstract Number: 0039 • ACR Convergence 2023
Enrichment of Rare Variants of Hemophagocytic Lymphohistiocytosis Genes in Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a complex inflammatory condition of childhood. It can be complicated by macrophage activation syndrome (MAS), a secondary form…Abstract Number: 0348 • ACR Convergence 2023
The Use of Ruxolitinib for Improved Disease Control in Systemic Juvenile Idiopathic Arthritis (sJIA) and Recurrent Macrophage Activation Syndrome (MAS)
Background/Purpose: The advent of biologics such as IL-1 receptor antagonists has dramatically improved outcomes for children with pediatric rheumatic diseases and hyperinflammation. However, there remains…Abstract Number: 031 • 2023 Pediatric Rheumatology Symposium
Multisystem Inflammatory Syndrome in Children Phenotypes Vary Between SARS-CoV-2 Variants
Background/Purpose: Multisystem Inflammatory Syndrome in Children (MIS-C) is a serious complication associated with COVID-19, presenting as a hyperinflammatory disorder characterized by fever and multiorgan dysfunction.…Abstract Number: 037 • 2023 Pediatric Rheumatology Symposium
Transcriptional Analysis of CD14+ Monocytes During Macrophage Activation Syndrome Highlights Role for Interferons and RNA Sensing in Monocytes
Background/Purpose: Macrophage activation syndrome (MAS), a form of secondary hemophagocytic lymphohistiocytosis (HLH), is a potentially fatal complication of rheumatic diseases. MAS is a dysfunctional hyperinflammatory…Abstract Number: 038 • 2023 Pediatric Rheumatology Symposium
HLA-DRB1*15 Alleles in Systemic Juvenile Idiopathic Arthritis with Lung Disease and Macrophage Activation Syndrome in Italy
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is characterized by unique clinical features and it is considered as a polygenic autoinflammatory disease. Macrophage activation syndrome (MAS)…
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