Abstract Number: 1149 • ACR Convergence 2020
Risk Score of Macrophage Activation Syndrome (MAS) in Patients with Systemic Juvenile Idiopathic Arthritis (sJIA)
Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of Systemic Juvenile Idiopathic Arthritis (sJIA) with a significant mortality. A score that identify sJIA…Abstract Number: 1150 • ACR Convergence 2020
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Background/Purpose: Macrophage Activation Syndrome (MAS) and secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm, in which IFNγ plays a pivotal role.…Abstract Number: 1151 • ACR Convergence 2020
Implementation and Initial Experience with a Screening Protocol for Inflammatory Hyperferritinemia
Background/Purpose: Macrophage activation syndrome (MAS) and hemophagocytic lymphohistiocytosis (HLH) epitomize a diverse and deadly group of inflammatory hyperferritinemic syndromes. Early biomarkers distinguishing these syndromes, especially…Abstract Number: 019 • 2020 Pediatric Rheumatology Symposium
Characterization of DOCK8 as a Novel Gene Associated with Cytokine Storm Syndrome
Background/Purpose: Cytokine storm syndromes (CSS), such as macrophage activation syndrome (MAS) and secondary hemophagocytic lymphohistiocytosis (HLH), are life threatening conditions that commonly present with unremitting…Abstract Number: 079 • 2020 Pediatric Rheumatology Symposium
Traditional Laboratory Parameters and New Biomarkers in Macrophage Activation Syndrome and Secondary Hemophagocytic Lymphohistiocytosis
Background/Purpose: Macrophage Activation Syndrome (MAS) and Secondary Hemophagocytic Lymphohistiocytosis (sHLH) are hyperinflammatory conditions caused by a cytokine storm. Prompt recognition and early treatment are essential…Abstract Number: 080 • 2020 Pediatric Rheumatology Symposium
Therapeutic Interferon Gamma Neutralization with Emapalumab in Patients with NRLC4- and CDC42-Associated Diseases Characterized by Recurrent and Severe Hemophagocytic Lymphohistiocytosis
Background/Purpose: Interferon gamma (IFNγ) is a pivotal mediator of HLH. Emapalumab (a fully human anti-IFNγ monoclonal antibody) is efficacious in patients with primary HLH. In…Abstract Number: 081 • 2020 Pediatric Rheumatology Symposium
Risk Score of Macrophage Activation Syndrome in Patients with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Macrophage Activation Syndrome (MAS) is a severe, life-threatening, complication of rheumatic diseases in childhood, particularly of systemic Juvenile Idiopathic Arthritis (sJIA), occurring in approximately…Abstract Number: 108 • 2020 Pediatric Rheumatology Symposium
A Multinational Study of Thrombotic Microangiopathy in Macrophage Activation Syndrome: A Dreadful Condition Which Is Likely Underrecognized
Background/Purpose: Macrophage activation syndrome (MAS) is a severe complication of rheumatologic conditions, mainly systemic juvenile idiopathic arthritis (sJIA), and is classified among the secondary forms…Abstract Number: 190 • 2020 Pediatric Rheumatology Symposium
Outcomes of an Evidence Based Guideline for the Treatment of Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome
Background/Purpose: Rapid identification of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) coupled with a multidisciplinary approach to management is essential to improve patient outcomes.…Abstract Number: 009 • 2020 Pediatric Rheumatology Symposium
Emapalumab (Anti-Interferon-Gamma Monoclonal Antibody) in Patients with Macrophage Activation Syndrome (MAS) Complicating Systemic Juvenile Idiopathic Arthritis (sJIA)
Background/Purpose: MAS is a severe complication of rheumatic diseases and occurs most frequently in patients with sJIA. Data from animal models and from observational studies…Abstract Number: 780 • 2019 ACR/ARP Annual Meeting
Changes in MiR-17-92 Cluster Expression Link Systemic Juvenile Idiopathic Arthritis, Monocyte-to-Macrophage Differentiation, and Interferon Regulation
Background/Purpose: MicroRNAs (miRNAs) are small noncoding RNAs which post-transcriptionally regulate gene expression. The miR-17-92 cluster is well characterized; its overexpression has been found to serve…Abstract Number: 811 • 2019 ACR/ARP Annual Meeting
Monomethyl Fumarate as a Novel Therapy for Macrophage Activation Syndrome: Mechanism of Action in an Animal Model
Background/Purpose: Macrophage activation syndrome (MAS) is a deadly systemic inflammatory condition marked by an increase in enzymes of iron metabolism including ferritin and heme-oxygenase 1…Abstract Number: 813 • 2019 ACR/ARP Annual Meeting
Monocyte and Macrophage Transcriptional Phenotypes in Systemic Juvenile Idiopathic Arthritis Reveal TRIM8 as a Mediator of IFNγ Hyperresponsiveness and Risk for Macrophage Activation Syndrome
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a severe and distinct subtype of childhood arthritis. Children with SJIA are at risk for macrophage activation syndrome…Abstract Number: 916 • 2019 ACR/ARP Annual Meeting
Development and Initial Validation of the MS Score for Diagnosis of Macrophage Activation Syndrome in Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Macrophage activation syndrome (MAS) is the most severe complication of systemic juvenile idiopathic arthritis (sJIA) and its adult equivalent, adult-onset Still’s disease (AOSD). Because…Abstract Number: 917 • 2019 ACR/ARP Annual Meeting
Systemic Juvenile Idiopathic Arthritis-Lung Disease: Characterization and Risk Factors
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (SJIA) is associated with a recently recognized albeit poorly defined and characterized lung disease (LD). Our objective is to describe…
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