Impact of Interleukin‐1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR / American College of Rheumatology (ACR) / Paediatric Rheumatology International Trials Organisation (PRINTO) 2016 Macrophage Activation Syndrome (MAS) Classification Criteria
Authors' conclusion
Although first-line anakinra in new-onset sJIA-SD has demonstrated high response rates, our data suggest the incidence of MAS in the first two years of disease is not reduced.
Study context
- Population
- Dutch patients with sJIA-SD treated with IL-1-targeted therapy (anakinra or canakinumab) with at least two years of follow-upcohorts of 54 and 66
- Outcome
- Applicability of the EULAR/ACR/PRINTO 2016 MAS classification criteria and incidence of MAS during IL-1-targeted therapy
Methodological detailsnationwide prospective cohort and intervention study, multicohort
- Nationwide prospective cohort and intervention study
- Multicohort
Main finding
As reported in the abstract
- Patients with MAS episodes while receiving IL-1-targeted treatment
- 22
- MAS episodes
- 29
- Patients with recurrent MAS episodes (not all while receiving IL-1 blockade)
- 7
- MAS episodes meeting 2016 criteria
- 28 of 29 MAS episodes (97%)
- Incidence of MAS in first two years, prospective cohort on first-line anakinra monotherapy
- 18% (12 of 66 patients, with 11 of 12 while receiving IL-1 inhibition)
Observational data: associations, not effects.
Further findings
Timing and triggers of MAS
- Half of MAS episodes occurred within three months after diagnosis
- Epstein-Barr virus was the most common identifiable trigger
- Incidence comparable to historical glucocorticoid-treated patients
Original publication
Impact of Interleukin‐1 Blockade on the Development of Macrophage Activation Syndrome in Still Disease: Incidence and Diagnostic Validity of the EULAR / ACR / PRINTO 2016 MAS Classification Criteria. Arthritis Rheumatol. Published 9 August 2025. doi:10.
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