Abstract Number: 2332 • 2017 ACR/ARHP Annual Meeting
Biomarkers for the Diagnosis and the Identification of Risk of Macrophage Activation Syndrome (MAS) in Systemic Juvenile Idiopathic Arthritis (sJIA)
Background/Purpose: We have recently reported high levels of IFNγ and of the IFNγ-related chemokines, (CXCL9 and CXCL10) in patients with MAS (1). Methods: Circulating levels…Abstract Number: 2333 • 2017 ACR/ARHP Annual Meeting
Adjudication of Infections from the Pharmacovigilance in Juvenile Idiopathic Arthritis Patients (PHARMACHILD) Treated with Biologic Agents and/or Methotrexate with a Focus on Opportunistic Infections
Background/Purpose: Pharmachild is a pharmacovigilance registry on children with JIA treated mainly with biologics ± methotrexate (MTX). Little evidence exists in literature about the role…Abstract Number: 2334 • 2017 ACR/ARHP Annual Meeting
Hypermethylation of NLRP3 Promoter Region Could be Responsible for Decreased Gene Expression, Inflammasome Malfunction and Gut Dysbiosis in Juvenile Spondyloarthritis Patients
Background/Purpose: Juvenile spondyloarthritis (jSpA) is a complex disease with both genetic and environmental factors contributing to the etiology. Recently obtained gene signatures in jSpA patients…Abstract Number: 2335 • 2017 ACR/ARHP Annual Meeting
Antiendothelial Cell Antibodies in Juvenile Dermatomyositis: A Proteomics-Based Approach
Background/Purpose: Juvenile dermatomyositis (JDM) is a systemic disorder of childhood characterized by muscle inflammation and vasculopathy. The mechanisms of the blood vessel injury in JDM…Abstract Number: 2336 • 2017 ACR/ARHP Annual Meeting
Expression of Myxovirus-Resistance Protein a: A Possible Marker of Muscular Disease Activity in Juvenile Dermatomyositis
Background/Purpose: Juvenile dermatomyositis (JDM) is a chronic autoimmune myopathy characterized by proximal muscle weakness and typical skin rashes. Type I interferon (IFN) gene expression in…Abstract Number: 2337 • 2017 ACR/ARHP Annual Meeting
Novel Serum Broad-Based Proteomic Discovery Analysis Identifies Proteins and Pathways Dysregulated in Juvenile Dermatomyositis (JDM) and Myositis Autoantibody Groups
Background/Purpose: Juvenile dermatomyositis (JDM) is a complex heterogeneous autoimmune disease. Myositis-specific autoantibodies (MSAs), present in up to 80% of JDM patients, help define distinct phenotypes…Abstract Number: 2338 • 2017 ACR/ARHP Annual Meeting
Plasma Exosomes from Children with Juvenile Dermatomyositis Are Taken up By Human Aortic Endothelial Cells and Are Associated with Altered Gene Expression in Those Cells
Background/Purpose: The pathology of juvenile dermatomyositis (JDM) is characterized by prominent vessel wall and perivascular inflammation. This feature of the disease has remained unexplained and…Abstract Number: 2339 • 2017 ACR/ARHP Annual Meeting
Expression of Type I and Type II Interferons Is Increased in Muscle Biopsies of Juvenile Dermatomyositis Patients and Related to Clinical and Histological Features
Background/Purpose: There is growing evidence for an involvement of interferons (IFNs) in the chronic inflammation that characterizes juvenile dermatomyositis (JDM). The aim of this study…Abstract Number: 2340 • 2017 ACR/ARHP Annual Meeting
Correlation of Type I Interferon Score and CXCL10 (C-X-C Motif Chemokine Ligand 10) with Cutaneous and Muscular Disease Activity in Juvenile Dermatomyositis Patients
Background/Purpose: Interferons (IFNs) seem to be important contributors in the pathogenesis of juvenile dermatomyositis (JDM). Our group previously reported that expression of both type I…Abstract Number: 2341 • 2017 ACR/ARHP Annual Meeting
The Interaction between Genetic Risk Factors and Age of Disease Onset in Juvenile Dermatomyositis
Background/Purpose: Juvenile dermatomyositis (JDM) is a rare, severe autoimmune disease characterized by muscle weakness and rash. Clinical features of JDM are heterogeneous, and can include…Abstract Number: 2342 • 2017 ACR/ARHP Annual Meeting
Transcriptomic Analysis Reveals Mitochondrial and Monocyte Dysfunctions Are Linked to the Interferonopathy of Juvenile Dermatomyositis
Background/Purpose: Although type I interferon (IFN1) and endoplasmic reticulum (ER) stress have been implicated in pathogenesis of juvenile dermatomyositis (JDM), little else is known about…Abstract Number: 2343 • 2017 ACR/ARHP Annual Meeting
A Genome-Wide Association Study Suggests the HLA Class II Region As the Major Susceptibility Locus for IgA Vasculitis
Background/Purpose: Immunoglobulin-A (IgA) vasculitis, also known as Henoch-Schöenlein purpura (HSP), is the most common type of primary small-sized blood vessel leukocytoclastic vasculitis in children, although…Abstract Number: 2344 • 2017 ACR/ARHP Annual Meeting
Cell-Bound Complement Activation Products As Markers of Disease Flares in Childhood Onset Systemic Lupus Erythematosus
Background/Purpose: Cell-bound complement activation products, CB-CAPs (C4d deposition on erythrocytes [EC4d], B lymphocytes [BC4d], reticulocytes [RC4d], platelets [PC4d], and C3d deposition on reticulocytes [RC3d]) are…Abstract Number: 2345 • 2017 ACR/ARHP Annual Meeting
Characterization of Adenosine Deaminase 2 Variants Identified in an International Pediatric Vasculitis Cohort
Background/Purpose: Deficiency of adenosine deaminase 2 (DADA2) is a recently recognized, autosomal recessive genetic disease. Patients present with various, early-onset systemic vascular and inflammatory manifestations,…Abstract Number: 2346 • 2017 ACR/ARHP Annual Meeting
Chemokine Ligand 9 (CXCL9) [Monokine Induced By Gamma Interferon (MIG)] As a Predictor of Active Disease Status in Localized Scleroderma
Background/Purpose: Localized scleroderma (LS) is a fibrotic autoimmune disease of the skin and underlying tissues which can lead to disfiguring sequlea, especially in childhood-onset. Untreated…