Publications by authors named "S J Vastert"

Unlabelled: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.

Methods: In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS).

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Objectives: Juvenile idiopathic arthritis (JIA) is the most common type of arthritis among children. It can cause joint pain and permanent physical damage, which affects mobility and daily activities. The EQ-5D-Y-3L self-report version has been validated in JIA, but the validity of EQ-5D-Y-5L remains unknown.

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Immune responses demand the rapid and precise regulation of gene protein expression. Splicing is a crucial step in this process; ~95% of protein-coding gene transcripts are spliced during mRNA maturation. Alternative splicing allows for distinct functional regulation, as it can affect transcript degradation and can lead to alternative functional protein isoforms.

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The cytokine storm syndrome (CSS) associated with systemic juvenile idiopathic arthritis (sJIA) has widely been referred to as macrophage activation syndrome (MAS). In this chapter, we use the term sJIA-associated CSS (sJIA-CSS) when referring to this syndrome and use the term MAS when referencing publications that specifically report on sJIA-associated MAS.

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Article Synopsis
  • The study aims to evaluate the current treatment approaches for macrophage activation syndrome (MAS) globally and identify inconsistencies in clinical practices.
  • A thorough literature review resulted in 57 relevant papers involving 1148 patients with MAS, primarily linked to systemic juvenile idiopathic arthritis (sJIA), systemic lupus erythematosus (SLE), and Kawasaki disease (KD).
  • Findings indicate high-dose glucocorticoids and certain targeted therapies, such as IL-1 and IFNγ inhibitors, are effective, particularly for sJIA-associated MAS, but highlight a need for more standardized research to improve treatment consistency for different conditions.
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