Abstract Number: 2417 • 2016 ACR/ARHP Annual Meeting
High Dimensional Interrogation of the T Cell Immunome in Polyarticular Juvenile Idiopathic Arthritis Patients
Background/Purpose: Clinical management of polyarticular JIA with anti-TNF-alpha has been met with moderate success, with up to 50% of patients demonstrating clinically meaningful efficacy. Concerns…Abstract Number: 2418 • 2016 ACR/ARHP Annual Meeting
Rnaseq Reveals Treatment-Associated Gene Expression Dynamics in Juvenile Idiopathic Arthritis CD4+ T Cells
Background/Purpose: While the field of pediatric rheumatology has made enviable strides in improving the lives of children with juvenile idiopathic arthritis (JIA), there is still…Abstract Number: 2419 • 2016 ACR/ARHP Annual Meeting
Single-Cell Analysis of CD163 mRNA and Protein Expression By Primeflow™ in Polarized Monocyte and Macrophage Populations
Background/Purpose: CD163 is involved in the regulation and resolution of innate inflammation and the removal of free hemoglobin from the blood via internalization of the…Abstract Number: 2420 • 2016 ACR/ARHP Annual Meeting
Investigating Genome-Wide Inbreeding Coefficients and Age of Diagnosis in a Multi-Ethnic Population of Childhood-Onset Systemic Lupus Erythematosus (cSLE)
Background/Purpose: Genetics plays an important role in the pathogenesis of systemic lupus erythematosus (SLE). Up to 20% of those affected with SLE are diagnosed in…Abstract Number: 2421 • 2016 ACR/ARHP Annual Meeting
Cell-Bound Complement Activation Products Correlate with Disease Activity in Childhood-Onset Systemic Lupus Erythematosus
Background/Purpose: Elevated levels of cell-bound complement activation products (C4d deposition on erythrocytes [EC4d] and B lymphocytes [BC4d], CB-CAPs) have been demonstrated to be sensitive and…Abstract Number: 2422 • 2016 ACR/ARHP Annual Meeting
Cyclic Amp, Erk5, and Transdifferentiation of Cardiac Fibroblasts in the Pathogenesis of Autoimmune Congenital Heart Block
Background/Purpose: Maternal autoantibodies (Ab) reactive with the Ro/La ribonucleoprotein complex are associated with the development of cardiac injury in a fetus passively exposed to these…Abstract Number: 2423 • 2016 ACR/ARHP Annual Meeting
Differential Interferon Score Expression in the Peripheral Blood in Mendelian Inflammatory Interferonopathies Versus Juvenile Dermatomyositis (JDM) Subtyped By Myositis Autoantibodies and Disease Activity
Background/Purpose: JDM is a complex autoimmune disease with an interferon (IFN) signature, with a reported correlation with disease activity. Clinical features vary among myositis-specific autoantibody…Abstract Number: 2424 • 2016 ACR/ARHP Annual Meeting
Novel Autoantigens for Anti- Endothelial Cell Antibodies in Pediatric Rheumatic Diseases Identified By Proteomics
Background/Purpose: Juvenile dermatomyositis (JDM), a systemic autoimmune vasculopathy, and juvenile idiopathic arthritis (JIA) are representative rheumatic diseases in children. However the pathogenesis of these diseases…Abstract Number: 2425 • 2016 ACR/ARHP Annual Meeting
The Vasculopathy of Juvenile Dermatomyositis (JDM); Evidence of Persistent Endothelial Injury, Hypercoagulability, Subclinical Inflammation and Increased Arterial Stiffness
Background/Purpose: Vasculopathy is considered central to the pathogenesis of Juvenile Dermatomyositis (JDM). The interplay between persistent JDM-vasculopathy, traditional cardiovascular risk factors, exposure to corticosteroids, and…Abstract Number: 2426 • 2016 ACR/ARHP Annual Meeting
Transcriptomic Analysis of Immune Subsets in Juvenile Dermatomyositis before and after Treatment Identifies Novel Pathways Involved in Pathogenesis
Background/Purpose: Although proximal muscle weakness and skin rash are the typical features of juvenile dermatomyositis (JDM), little is known about disease pathogenesis, why other features…Abstract Number: 2427 • 2016 ACR/ARHP Annual Meeting
Identification of Biomarkers Using Tear Proteomics in Children with Chronic Anterior Uveitis
Background/Purpose: Children with juvenile idiopathic arthritis (JIA) are at risk for anterior uveitis which can lead to ocular complications and vision loss. The ANA is…Abstract Number: 2428 • 2016 ACR/ARHP Annual Meeting
Autoantibody Diversity in Pediatric Patients Undergoing Evaluation for Autoimmune Encephalitis: A Retrospective Investigation
Background/Purpose: Antibodies directed against N-methyl-D-aspartate type glutamate receptor (NMDAR), voltage-gated potassium channel (VGKC) or glutamic acid decarboxylase (GAD65) are frequently associated with autoimmune encephalitis (AE)…Abstract Number: 2429 • 2016 ACR/ARHP Annual Meeting
Mechanisms for the Development of Lung Fibrosis in Sting-Associated Vasculopathy with Onset in Infancy (SAVI)
Background/Purpose: STING-Associated Vasculopathy with Onset in Infancy (SAVI) is a monogenic autoinflammatory interferonopathy caused by gain-of-function mutations in TMEM173/STING, a nucleic acid sensor adaptor linked…Abstract Number: 2430 • 2016 ACR/ARHP Annual Meeting
Mutations in the Tyrosine-Protein Kinase Lyn Cause an Early-Onset Neutrophilic Vasculitis Syndrome
Background/Purpose: Tyrosine-protein kinase Lyn is a Src-family tyrosine kinase expressed by hematopoietic and non-hematopoietic cell types. Phosphorylation of a tyrosine residue at position 508 renders…Abstract Number: 2431 • 2016 ACR/ARHP Annual Meeting
Characterization of Innate Immune Cells in Patients with the Interferon-Mediated Autoinflammatory Diseases Sting Associated Vasculopathy with Onset in Infancy (SAVI) and Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and Elevated Temperature (CANDLE)
Background/Purpose: We recently described two rare autoinflammatory interferonopathies, STING Associated Vasculopathy with Onset in Infancy (SAVI) and Chronic Atypical Neutrophilic Dermatosis with Lipodystrophy and…
