Pediatric rheumatology

Systemic juvenile idiopathic arthritis and adult-onset Still's disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still's disease

Ann Rheum DisPublished Systematic reviews and meta-analyses

Authors' conclusion

Our results argue for a continuum between sJIA and AOSD.

Study context

Population
Cohort studies comparing systemic juvenile idiopathic arthritis (sJIA) vs adult-onset Still's disease (AOSD) with at least 20 patients per group (SR1); biomarker studies in both diseases (SR2); diagnostic biomarkers for macrophage activation syndrome (MAS) (SR3)8 studies in SR1
Participants
1,010 participants
Comparison
sJIA vs AOSD
Outcome
Similarity in clinical manifestations and laboratory findings between sJIA and AOSD
Methodological detailsthree systematic reviews, meta-analyses of proportions, risk of bias: adapted Hoy scale (SR1), QUADAS-2 (SR2, SR3), Medline (PubMed), Embase and Cochrane Library systematically searched
  • Three systematic reviews
  • Meta-analyses of proportions
  • Risk of bias: adapted Hoy scale (SR1), QUADAS-2 (SR2, SR3)
  • Medline (PubMed), Embase and Cochrane Library systematically searched

Pooled result

As reported in the abstract

Pooled prevalence of clinical manifestations, complications and biological findings
  • Pooled prevalence of clinical manifestations did not differ between sJIA and AOSD, except myalgia, sore throat and weight loss, more frequent in AOSD than sJIA
  • Except for AA amyloidosis, more frequent in sJIA than AOSD, prevalence of complications did not differ
  • Prevalence of biological findings did not differ

Further pooled results

Diagnostic biomarkers (SR2, 33 studies)

Ferritin, S100 proteins and IL-18 were the most frequently used diagnostic biomarkers, with similar diagnostic performance

Biomarkers for MAS diagnosis (SR3, 10 studies)

Novel biomarkers such as IL-18, C-X-C motif ligand 9, adenosine deaminase 2 activity and activated T cells seemed promising

Original publication

Systemic juvenile idiopathic arthritis and adult-onset Still's disease are the same disease: evidence from systematic reviews and meta-analyses informing the 2023 EULAR/PReS recommendations for the diagnosis and management of Still's disease. Ann Rheum Dis. Published 14 November 2024. doi:10.1136/ard-2024-225853

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