Abstract Number: 1768 • 2019 ACR/ARP Annual Meeting
A First-in-class Selective and Potent IRAK4 Degrader Demonstrates Robust in Vitro and in Vivo Inhibition of TLR/IL-1R Activation and Inflammation
Background/Purpose: IL-1R/TLR activation plays a central role in the pathophysiology of multiple autoimmune and inflammatory diseases driven by the IL-1 family of cytokines and by…Abstract Number: 2156 • 2019 ACR/ARP Annual Meeting
Expanding the Phenotypic and Genotypic Spectrum in Yao Syndrome
Background/Purpose: Yao syndrome (YAOS, OMIM 617321), formerly termed nucleotide-binding, oligomerization domain 2(NOD2)-associated autoinflammatory disease, has become increasingly recognized. The disease has been reported in America,…Abstract Number: 2698 • 2019 ACR/ARP Annual Meeting
Reasons for Initiation of Canakinumab of Patients with Systemic Juvenile Idiopathic Arthritis: A Retrospective Medical Chart Review from the United States
Background/Purpose: Systemic juvenile idiopathic arthritis (SJIA) is a rare autoinflammatory disease characterized by fever and arthritis, often accompanied by rash. Canakinumab (CAN) was approved in…Abstract Number: 2903 • 2019 ACR/ARP Annual Meeting
Cryopyrin-Associated Periodic Syndrome Treated with Canakinumab – Long-Term Follow-up Data Documents Sustained Safety and Remission
Background/Purpose: Targeting the interleukin(IL)-1 pathway with anti-IL-1 drugs is a treatment option in patients with autoinflammatory diseases like monogenic periodic fever syndromes. The study aims…Abstract Number: 772 • 2019 ACR/ARP Annual Meeting
The Study of the Novel G87V Mutation in the TNFRSF1A Gene Identified in a Family with TNF Receptor-Associated Periodic Syndrome (TRAPS)
Background/Purpose: TNF Receptor-Associated Periodic Syndrome (TRAPS) is one of the autoinflammatory diseases. TRAPS is caused by heterozygous mutations in the TNFRSF1A gene. Although more than…Abstract Number: 799 • 2019 ACR/ARP Annual Meeting
Application of the Autoinflammatory Disease Activity Index (ADDI) to a Cohort of Patients in a Tertiary Hospital
Background/Purpose: Autoinflammatory diseases (AIDs) cause chronic systemic inflammation that can damage multiple organs. Recently, the ADDI index has been developed and validated in the four…Abstract Number: 926 • 2018 ACR/ARHP Annual Meeting
Truncating Mutations in SAMD9L Cause an Early-Onset Immune-Dysregulatory Syndrome of Neutrophilic Panniculitis, Interstitial Lung Disease and Cytopenias
Background/Purpose: The Sterile Alpha Motif Domain Containing 9 Like protein that is encoded by SAMD9L plays a role in endosome fusion, and deletions (haploinsufficiency) of…Abstract Number: 1321 • 2018 ACR/ARHP Annual Meeting
Clinical and Genetic Analysis of the Patients Mimicking Familial Mediterranean Fever
Clinical and genetic analysis of the patients mimicking familial Mediterranean feverBackground/Purpose: Familial Mediterranean fever (FMF) is an auto-inflammatory disease characterized by recurrent episodes of fever…Abstract Number: 1412 • 2018 ACR/ARHP Annual Meeting
A Systematic Literature Review of Efficacy and Safety of Current Therapies for the Treatment of Hyperimmunoglobulinemia D Syndrome and TNF Receptor-Associated Periodic Syndrome
Background/Purpose: Hyperimmunoglobulinemia D syndrome (HIDS), also known as mevalonate kinase deficiency (MKD), and TNF receptor-associated periodic syndrome (TRAPS) are rare auto-inflammatory diseases grouped as periodic…Abstract Number: 1415 • 2018 ACR/ARHP Annual Meeting
Emapalumab, an Anti-Interferon Gamma Monoclonal Antibody in Two Patients with NLRC4-Related Disease and Severe Hemophagocytic Lymphohistiocytosis (HLH)
Background/Purpose: Interferon gamma (IFNγ) plays a pathogenic role in primary and secondary HLH. An ongoing phase 2/3 trial with emapalumab in primary HLH provides encouraging…Abstract Number: 1426 • 2018 ACR/ARHP Annual Meeting
The Utilization of S100 Proteins Testing in Pediatric Rheumatology Patients in a Tertiary Care Institution and Implications for Care
Background/Purpose: S100 proteins are calcium-binding proteins of increasing value as biomarkers in various inflammatory conditions (e.g. autoinflammatory diseases, vasculitides, inflammatory bowel disease). The two highly…Abstract Number: 1911 • 2018 ACR/ARHP Annual Meeting
The Expanding Clinical Spectrum of Patients with Deficiency of Adenosine Deaminase 2 (DADA2)
Background/Purpose: The deficiency of adenosine deaminase 2 (DADA2) was initially described in 2014 in 2 reports: one emphasizing early-onset lacunar strokes, livedoid rash and intermittent…Abstract Number: 2023 • 2018 ACR/ARHP Annual Meeting
Different Patterns of Interferon-Response-Gene Expression May Elucidate Different Pathomechanisms That Drive IFN-Response-Gene Activation in Patients with Presumed IFN-Mediated Autoinflammatory Diseases
Background/Purpose: Many infants and children with early-onset autoinflammatory diseases are mutation-negative for genetically known autoinflammatory diseases. Recent data suggest a role for Type-I interferon dysregulation…Abstract Number: 2279 • 2018 ACR/ARHP Annual Meeting
Therapeutic Value of Canakinumab in Patients with Yao Syndrome
Background/Purpose: Yao syndrome (YAOS, OMIM 617321), formerly termed nucleotide-binding, oligomerization domain 2(NOD2)-associated autoinflammatory disease, is characterized by periodic fever, dermatitis, arthritis, and swelling of the…Abstract Number: 2855 • 2018 ACR/ARHP Annual Meeting
Expanding the Phenotype: New Variant in the IL1RN-Gene Associated with Late Onset and Atypical Presentation of Dira
Background/Purpose: Deficiency of the Interleukin-1 receptor antagonist (DIRA) is an autoinflammatory disease of infancy characterized by severe systemic inflammation with bone and skin involvement. This…
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