Abstract Number: 1143 • ACR Convergence 2023
Nationwide Analysis of Adult-Onset Still’s Disease with and Without Hemophagocytic Lymphohistiocytosis
Background/Purpose: Adult-Onset Still's Disease (AOSD) commonly manifests with fever, arthritis, rash, liver dysfunction, lymphadenopathy, and hematologic abnormalities. AOSD is also associated with Hemophagocytic Lymphohistiocytosis (HLH).…Abstract Number: 1660 • ACR Convergence 2023
Still’s Disease Patients with High Interferon-stimulated Gene Expression Have Enrichment of Rare, de Novo and Recessive Protein Altering Variants in Innate Immune Pathways
Background/Purpose: Still’s disease (systemic juvenile idiopathic arthritis in children, adult-onset Still’s disease in adults) is an enigmatic inflammatory condition that affects people of all ages.…Abstract Number: 1932 • ACR Convergence 2023
The Association Between Initial Ferritin and Complications from Adults Onset Stills Disease and Intensive Care Unit Admission
Background/Purpose: Adult Onset Still's Disease (AOSD) is an autoinflammatory disease characterized by quotidian fever, arthralgia, and a salmon colored rash. Due to its rarity, there…Abstract Number: 2473 • ACR Convergence 2023
A Consensus Based Algorithm to Screen for Lung Disease in Children with Systemic Juvenile Idiopathic Arthritis
Background/Purpose: Over the last decade, lung disease (LD) has become an increasingly recognized complication of systemic juvenile idiopathic arthritis (sJIA). Children with sJIA-LD may be…Abstract Number: 0758 • ACR Convergence 2023
Clinical and Biological Characteristics of Children and Adults Affected with Still’s Disease: A Systematic Review and Meta-analysis Informing the 2023 EULAR/PReS Recommendations for the Diagnosis and Management of Systemic Juvenile Idiopathic Arthritis and Adult-onset Still’s Disease
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD) are part of the non-familial (sporadic) systemic inflammatory disorders and are frequently mentioned as…Abstract Number: 0759 • ACR Convergence 2023
Derivation and Validation of Four Patient Clusters in Still’s Disease, Results from GIRRCS AOSD-study Group and AIDA Network Still Disease Registry
Background/Purpose: Still's disease is a rare inflammatory disorder, these patients have a highly heterogeneous disease according to age of onset, clinical presentation, presence of life-threatening…Abstract Number: 0760 • ACR Convergence 2023
Treatment Patterns and Outcomes in Patients with Macrophage Activation Syndrome Secondary to Still’s Disease Treated with Emapalumab: The REAL-HLH Study
Background/Purpose: Macrophage activating syndrome (MAS) is a rare, potentially fatal complication of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD). MAS (a form…Abstract Number: 036 • 2023 Pediatric Rheumatology Symposium
Predictive Factors of Long-lasting Remission Following Anakinra Withdrawal in Patients with Systemic Juvenile Idiopathic Arthritis After Achievement of Clinical Inactive Disease
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a rare autoinflammatory disease of unknown etiology. Several uncontrolled studies showed that early treatment with anakinra is associated…Abstract Number: 097 • 2023 Pediatric Rheumatology Symposium
Survey of Covid-19 Immunization and Infection in Patients with Systemic Juvenile Idiopathic Arthritis and Adult Onset Still’s Disease
Background/Purpose: Immunization is one of the most important tools for the control of the Covid-19 pandemic. The safety and effectiveness of the Covid-19 immunizations have…Abstract Number: 113 • 2023 Pediatric Rheumatology Symposium
Proportion of Patients with a Polyphasic Disease Course in Systemic-onset Juvenile Idiopathic Arthritis May Be Higher in the Age of Cytokine Inhibitors
Background/Purpose: Systemic-onset juvenile idiopathic arthritis (sJIA) is a pediatric autoinflammatory condition, known for significant variability between patients in its severity and long-term outcomes. The classification…Abstract Number: L12 • ACR Convergence 2022
First Line Treatment Using Recombinant IL-1Receptor Antagonist in New Onset Systemic Juvenile Idiopathic Arthritis Is an Effective Treatment Strategy, Irrespective of HLA DRB1 Background
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a severe subtype of JIA. Recently, interstitial lung disease (SJIA-LD) has been reported as a severe complication of…Abstract Number: 0562 • ACR Convergence 2022
Large-Scale Targeted Sequencing Study Links Systemic Juvenile Idiopathic Arthritis with Rare Variants of MEFV, LYST, STXBP2, UNC13D
Background/Purpose: Systemic juvenile idiopathic arthritis (sJIA) is a genetically complex inflammatory condition. It can be marked by severe systemic inflammation that resembles the hereditary periodic…Abstract Number: 0826 • ACR Convergence 2022
Treatment Pattern and Changes in Oral Glucocorticoid Dose After Tocilizumab Treatment in Patients with Adult Still’s Disease: An Analysis of a Japanese Claims Database
Background/Purpose: Adult Still's disease (ASD), including adult-onset Still's disease and carry-over systemic juvenile idiopathic arthritis (sJIA), is treated with glucocorticoids (GC) with or without immunosuppressive…Abstract Number: 0829 • ACR Convergence 2022
Adult Onset Still’s Disease: A Retrospective Study of Objective Manifestations and Outcomes
Background/Purpose: Adult Onset Still's Disease (AOSD) is a complex and rare inflammatory disease with life threatening complications. Due to its rarity and wide ranging clinical…Abstract Number: 0863 • ACR Convergence 2022
Effect of Glucocorticoids on Patient Reported Outcomes in Patients Started on a Biologic Consensus Treatment Plan for the ‘First Line Options for Systemic JIA Treatment’ (FROST) Study
Background/Purpose: Systemic Juvenile Idiopathic Arthritis (sJIA) is a systemic autoinflammatory disease characterized by high fevers, rash and arthritis. Current treatment regimens often involve biologic (anti-IL-1…
ACR Meeting Abstracts