Abstract Number: 1125 • ACR Convergence 2023
A Rheumatologic Clinical Profile of the VEXAS Syndrome: Results from a Survey Conducted Among Rheumatologic Units of 126 Hospitals Across Spain
Background/Purpose: The newly described VEXAS syndrome is a very heterogenous disease with rheumatologic and hematologic manifestations, caused by somatic mutations affecting UBA1 gene, that still…Abstract Number: 1913 • ACR Convergence 2023
Neurosarcoidosis Disease Epidemiology. University Hospital in Northern Spain 1999-2019
Background/Purpose: Neurosarcoidosis (NS) is a serious and relative uncommon complication of sarcoidosis. Data on incidence is scarce and varies worldwide. Our objective is toestimate NS…Abstract Number: 2175 • ACR Convergence 2023
Real-World Drug Persistence of GP2015, an Etanercept Biosimilar, in Patients with Rheumatoid Arthritis: Results from the Multi-Country COMPACT Study
Background/Purpose: COMPACT is a multi-country, non-interventional study that evaluated the drug persistence, effectiveness, safety and patient-reported outcomes in patients with rheumatoid arthritis (RA), axial spondyloarthritis…Abstract Number: 0260 • ACR Convergence 2023
Phenotypes of the Patients with More Than One Autoinflammatory Gene Variant: Classified Diseases and Mixed Autoinflammatory Disorders (MAID)
Background/Purpose: Background: Systemic autoinflammatory disorders (SAIDs) are including a group of diseases, which are associated with genetic variations resulting in dysregulation of innate immunity and…Abstract Number: 1129 • ACR Convergence 2023
Accuracy and Performance Characteristics of Administrative Codes for the Diagnosis of Autoinflammatory Syndromes: A Discovery and Validation Study
Background/Purpose: Autoinflammatory syndromes (AIS), a group of rare rheumatic diseases driven by the innate immune system, remain understudied due to the lack of prospective cohorts.…Abstract Number: 1916 • ACR Convergence 2023
Clinical Features of the Patients with NLRP1 Gene Variants and a Systemic Autoinflammatory Phenotype
Background/Purpose: Although NLRP1 was the first identified member of the NOD-like receptors family, its role as a sensor of pathogen- or damage-associated signals and its…Abstract Number: 2477 • ACR Convergence 2023
Anifrolumab Normalizes the Type I Interferon Signature in a Cohort of Patients with Type I Interferonopathies
Background/Purpose: Autoinflammatory Type I Interferonopathies (IFNopathies) include SAVI (STING-associated vasculopathy with onset in infancy), CANDLE/PRASS (Chronic atypical neutrophilic dermatosis, with lipodystrophy and elevated temperature), and…Abstract Number: 0269 • ACR Convergence 2023
Acute Kidney Injury and Plasma Cell-Rich Acute Interstitial Nephritis in VEXAS Syndrome: An Under-recognized Disease Feature
Background/Purpose: VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) is a recently identified autoinflammatory disease with a large variety of disease manifestations. While recurrent fever,…Abstract Number: 1132 • ACR Convergence 2023
Preclinical Profiles of FZ007-119, a Highly Potent and Selective Tyk2 Inhibitor, for the Treatment of Immune Mediated Inflammatory Diseases
Background/Purpose: Tyrosine Kinase 2 (TYK2) is a signaling protein within the Janus kinase (JAK) family. It plays a crucial role in transmitting signals from pro-inflammatory…Abstract Number: 1918 • ACR Convergence 2023
Nationwide Analysis of Predictors of Sarcoid Inpatient Mortality
Background/Purpose: Sarcoidosis is multisystem autoimmune disease characterized by noncaseating granulomas which can result in significant morbidity and mortality. This study aims to identify variables associated…Abstract Number: 2505 • ACR Convergence 2023
Discovery of First-in-Class IRAK4 Scaffolding Inhibitors for the Treatment of Inflammatory Disorders
Background/Purpose: Interleukin-1 receptor-associated kinase 4 (IRAK4), is a key node that mediates inflammatory signaling in response to activation of toll-like receptor (TLR) and IL-1 receptor…Abstract Number: 0006 • ACR Convergence 2023
Preclinical Characterization of a Novel anti-CD40 Antagonist Antibody-glucocorticoid Conjugate with Superior Preclinical Efficacy and Favorable Safety Profile
Background/Purpose: Glucocorticoids (GCs) are the first-line anti-inflammatory treatment for many autoimmune diseases, but long-term systemic administration of GCs is associated with unwanted side effects, which…Abstract Number: 0275 • ACR Convergence 2023
No Cumulative Effect of Infection Rates in Children Receiving Long-term Canakinumab Treatment in Autoinflammatory Periodic Fever Syndromes − Data from the RELIANCE Registry
Background/Purpose: Autoinflammatory diseases (AID) have been treated safely and effectively with the interleukin-1β inhibitor canakinumab (CAN) in controlled trials and routine clinical practice. The most…Abstract Number: 1136 • ACR Convergence 2023
Challenges in Diagnosing VEXAS Syndrome: Delayed Diagnosis, Misdiagnosis, and Associations with Specific Gene Mutations
Background/Purpose: The newly described VEXAS syndrome is a very heterogenous disease with rheumatologic and hematologic manifestations, caused by somatic mutations affecting UBA1 gene, that still…Abstract Number: 1921 • ACR Convergence 2023
Clinical Spectrum of VEXAS Syndrome in a Rheumatology Department
Background/Purpose: VEXAS (vacuoles, E1 activating enzyme, X-linked, auto-inflammatory, somatic) syndrome is a clinically serious and potentially fatal adult-onset disease caused by somatic mutations in the…
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