Abstract Number: 023 • 2023 Pediatric Rheumatology Symposium
Effect of Type 1 Interferons and JAK Inhibitors on Gene Expression in Bioengineered Pediatric Skeletal Muscle
Background/Purpose: Genetic studies of new-onset juvenile dermatomyositis (JDM) exhibit elevation of Type 1 interferons (IFN 1) IFNα and IFNβ in blood, skin, and muscle. To…Abstract Number: 075 • 2023 Pediatric Rheumatology Symposium
Emapalumab Treatment Followed by Hematopoietic Stem Cell Transplantation in Systemic Juvenile Idiopathic Arthritis Complicated by Recurrent Macrophage Activation Syndrome
Background/Purpose: Macrophage activation syndrome (MAS) is a life-threatening complication of different rheumatic diseases, particularly of systemic juvenile idiopathic arthritis (sJIA).Methods: We report the case of…Abstract Number: 080 • 2023 Pediatric Rheumatology Symposium
Analysis of Proteasomal Activity – a Potential Diagnostic Tool for Proteasome-associated Autoinflammatory Syndromes (PRAAS)
Background/Purpose: Interferonopathies are a recently recognized group of genetic syndromes associated with uncontrolled activation of interferon. PRAAS (proteasome-associated autoinflammatory syndromes) is an interferonopathy caused by…Abstract Number: 0158 • ACR Convergence 2022
Effect of Type I IFN on Mitochondria and Muscle Weakness in Myositis
Background/Purpose: Muscle weakness is a hallmark of autoimmune myositis. The mechanisms that contribute to muscle weakness are currently unknown. The observed ineffectiveness after immunomodulatory treatment…Abstract Number: 0663 • ACR Convergence 2022
Anti-Erythrocyte Antibodies in Childhood Onset Systemic Lupus Erythematosus
Background/Purpose: Systemic Lupus Erythematosus (SLE) is a disease characterized by the presence of auto-antibodies, immune complex deposition, and a robust type 1 interferon signature. It…Abstract Number: 1745 • ACR Convergence 2022
Effects of Excess IL-18 in Mixed Inflammatory Environments
Background/Purpose: Interleukin 18 (IL-18) is an inflammasome-activated, IL-1 family cytokine that canonically induces interferongamma (IFNg). IL-18 activity is potently inhibited by a soluble, IFNg-inducible antagonist,…Abstract Number: 0324 • ACR Convergence 2022
Comparison of NCounter® and BioFire® Technologies for the Measurement of Type I Interferon Signature
Background/Purpose: It is known that the expression of type I Interferon (IFN) genes is upregulated in systemic lupus erythematosus (SLE). In this study, we determined…Abstract Number: 0668 • ACR Convergence 2022
Differential Expression of Interferon Related Genes in SLE Patients of Asian and European Ancestries Abstract
Background/Purpose: Interferon has been shown to play a role in the pathogenesis of SLE, but insufficient studies have been conducted into the differences in interferon…Abstract Number: 1862 • ACR Convergence 2022
Lenabasum Reduces IFNγ and pIRF3 in Dermatomyositis Skin: Biomarker Results from a Double-Blind Phase 3 International Randomized Controlled Trial
Background/Purpose: Dermatomyositis (DM) is an autoimmune connective tissue disease that primarily affects the skin and lung; few effective treatment options are available. Lenabasum is a…Abstract Number: 0359 • ACR Convergence 2022
Attainment of Remission with Anifrolumab: A Post Hoc Analysis of Pooled TULIP-1 and TULIP-2 Datasets
Background/Purpose: In patients with SLE, achieving remission is a treat-to-target goal. Remission is associated with lower rates of hospitalization and damage accrual and better quality…Abstract Number: 0669 • ACR Convergence 2022
Understanding How Type I Interferon Modulates Langerhans Cell ADAM17 to Promote Photosensitivity in Lupus
Background/Purpose: Photosensitivity is a hallmark symptom of lupus erythematosus (LE), in which patients develop inflammatory skin lesions in response to ultraviolet radiation (UVR). In examining…Abstract Number: 1879 • ACR Convergence 2022
Analysis of the Association Between the Atrophic Factors Tripartite Motif Containing (TRIM) 63 and Atrogin-1 and the Clinical and Inflammatory Features of Patients with Idiopathic Inflammatory Myopathies
Background/Purpose: Muscle atrophy is mediated by the ubiquitination of myofilaments by two ubiquitin ligases called Tripartite Motif Containing (TRIM) 63 and Atrogin-1, which are induced…Abstract Number: 0360 • ACR Convergence 2022
Longitudinal Variation of Proteomic Biomarkers That Correlate with Efficacy Endpoints: Results from a Phase 3 Trial of Anifrolumab in Moderate to Severe Systemic Lupus Erythematosus
Background/Purpose: Phase 2/3 clinical trials in patients with moderate to severe SLE have demonstrated that anifrolumab, a monoclonal antibody blocking IFNAR1, produced better clinical outcomes…Abstract Number: 0740 • ACR Convergence 2022
Real-World Demographics, Clinical Characteristics, and Treatment Patterns of Patients Treated with Emapalumab for Secondary Hemophagocytic Lymphohistiocytosis in the United States: The REAL-HLH Study
Background/Purpose: Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening, hyperinflammatory syndrome caused by overproduction of proinflammatory cytokines, e.g., interferon gamma (IFNγ). Secondary HLH (sHLH), a subtype…Abstract Number: 1931 • ACR Convergence 2022
NOD2 Mutations Are Associated with Upregulated Type 1 Interferon Gene Expression and Development of Granulomatous Hepatitis in Children with Autoimmune Hepatitis
Background/Purpose: Mutations in the gene NOD2 encoding the nucleotide-binding oligomerization domain-containing protein 2 (NOD2) which controls innate responses to LPS have been linked to Blau…
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