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: 1152 • ACR Convergence 2020
IL-18: A Biomarker That Reflects Disease Activity, Could It Be the Next Disease Activity Measure in Systemic Juvenile Idiopathic Arthritis?
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a childhood arthritis with prominent innate immune activity. Disease presentation and flares could largely mimic infections with fever…Abstract Number: 1153 • ACR Convergence 2020
Trends in Timing of Biologic Use for Treatment of Systemic Juvenile Idiopathic Arthritis in the CARRA Registry
Background/Purpose: The treatment of systemic juvenile idiopathic arthritis (SJIA) has changed dramatically over the past decade, associated with overall improvement in functional outcomes. There may…Abstract Number: 1154 • ACR Convergence 2020
Distinct Gene Signature Predicts Strong Clinical Responses to Canakinumab in Children with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Canakinumab is a human anti-IL1β blocking agent that effectively neutralizes IL1β mediated signaling and is used to treat diseases such as systemic juvenile idiopathic…Abstract Number: 1155 • ACR Convergence 2020
Long-term Safety Profile of Anakinra in Patients with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: To evaluate the long-term safety profile of anakinra in patients with systemic juvenile idiopathic arthritis (sJIA)Methods: Data from patients with sJIA according to the…Abstract Number: 1156 • ACR Convergence 2020
Comparison of Immunological Biomarkers and Lung Histology in Patients with Elevated IL18 – Pulmonary Alveolar Proteinosis and Recurrent Macrophage Activation Syndrome (IL-18PAP-MAS) and Other Inflammatory Lung Diseases
Background/Purpose: Recently, pulmonary alveolar proteinosis (PAP) and recurrent macrophage activation syndrome (MAS) have been reported in rare patients (pts) with systemic juvenile idiopathic arthritis (SJIA)…Abstract Number: 1157 • ACR Convergence 2020
Health and Socioeconomic Outcomes in a Neonatal-Onset Multisystem Inflammatory Disease (NOMID) Cohort Followed for a Median of Fifteen Years
Background/Purpose: Patients (pts) with NOMID have systemic inflammation and organ damage such as sensorineural hearing loss, hydrocephalus, optic nerve atrophy and growth plate defects. IL-1 blocking…Abstract Number: 1158 • ACR Convergence 2020
Clinical Features and Outcomes in STING-Associated Vasculopathy with Onset in Infancy (SAVI)
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is an autoinflammatory interferonopathy caused by gain-of-function mutations in STING1, characterized by peripheral vasculopathy and interstitial lung…Abstract Number: 1159 • ACR Convergence 2020
Novel STING1 Mutations Including in the Transmembrane Linker Region Cause STING-associated Vasculopathy with Onset in Infancy (SAVI)
Background/Purpose: STING-associated vasculopathy with onset in infancy (SAVI) is an autoinflammatory disease caused by gain-of-function (GOF) mutations in STING1/TMEM173 that encodes stimulator of interferon genes,…Abstract Number: 1160 • ACR Convergence 2020
Treatment Intensity and Impact on Bone Lesion Evolution and Distribution Patterns in Severe Chronic Recurrent Multifocal Osteomyelitis
Background/Purpose: To compare bone lesion evolution and bone lesion distribution patterns identified by whole body magnetic resonance imaging (WB-MRI) by treatment intensity in patients with…Abstract Number: 1161 • ACR Convergence 2020
Perspectives of Radiologist Physicians in the Imaging of Chronic Nonbacterial Osteomyelitis
Background/Purpose: Radiological imaging is integral to the diagnosis of chronic nonbacterial osteomyelitis (CNO) and has been included as a central component in suggested diagnostic criteria…Abstract Number: 1162 • ACR Convergence 2020
Comparison of Clinicopathologic and Imaging Features Between Chronic Nonbacterial Osteomyelitis and Its Mimickers: A Multi-national 450 Case-Control Study
Background/Purpose: Chronic nonbacterial osteomyelitis (CNO)/chronic recurrent multifocal osteomyelitis (CRMO) predominantly affects children and young adults. Classification criteria are not available and diagnostic criteria that have…Abstract Number: 1163 • ACR Convergence 2020
Predictors of Colchicine Response in Patients with Undefined Systemic Autoinflammatory Diseases
Background/Purpose: Systemic autoinflammatory diseases (SAIDs) result from dysregulation of the innate immune system. Many patients with SAIDs have specific mutations that lead to the release…
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