Pediatric rheumatology

Traditional laboratory parameters and IFNγ-related biomarkers in the diagnosis and management of macrophage activation syndrome (MAS)

Semin Arthritis RheumPublished Diagnostic accuracy study

Authors' conclusion

These findings highlight the potential role of CXCL9 measurement in supporting the diagnosis and guiding the therapeutic management of patients with MAS in clinical practice.

Study context

Population
Patients with MAS secondary to Still's disease and patients with active pediatric Still's disease, six Italian centres
Participants
41 vs 24
Comparison
MAS secondary to Still's disease vs active pediatric Still's disease
  • MASn = 41
  • Active SDn = 24reference
Outcome
Diagnostic and prognostic significance of traditional laboratory parameters of hyperinflammation and of IFNγ-related biomarkers (IL-18, CXCL9, neopterin) in MAS
Methodological detailsmulticentre
  • Multicentre

Performance

As reported in the abstract

Discrimination of MAS from active SD; prognosis of severe MAS · baseline

Prognostic score (baseline CXCL9 + ferritin, platelet count, fibrinogen, LDH) for severe MAS: sensitivity

100 %

Prognostic score for severe MAS: specificity

74 %

Further findings

Risk of failing to achieve MAS remission in ≤2 months, by IFNγ-related biomarker levels at T1 (contingency analysis) · T1 (5-15 days from baseline)

OR for failing to achieve MAS remission in ≤2 months
CXCL9 >830 pg/ml at T1Significant risk as printed9.3
IL-18 >83,000 pg/ml at T1Significant risk as printed5.4

Original publication

Traditional laboratory parameters and IFNγ-related biomarkers in the diagnosis and management of MAS. Semin Arthritis Rheum. Published 12 August 2025. doi:10.1016/j.semarthrit.2025.152812

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