2016 sJIA-MAS criteria and a step-up therapeutic approach in KD-MAS: insights from a combined cohort and literature review
Authors' conclusion
The 2016 sJIA-MAS criteria demonstrate the highest proportion fulfilling criteria in our cohort for KD-MAS.
Study context
- Population
- Children with Kawasaki disease-associated macrophage activation syndrome (KD-MAS), single centre; mean age 3.72 yearsKD-MAS of 3786 KD
- Participants
- 22 children
- Exposure
- HLH-2004, HLH-2009 and 2016 sJIA-MAS classification criteria in clinician-diagnosed KD-MAS
- Outcome
- Clinician-diagnosed KD-MAS cases fulfilling each classification framework
Methodological detailssingle-centre, with literature review
- Single-centre
- With literature review
Main finding
As reported in the abstract
HLH-2004
14 of 22
events
HLH-2009
18 of 22
events
2016 sJIA-MAS
20 of 22
events
- Met all three frameworks
- 11
Observational data: associations, not effects.
Further findings
Median
| Outcome | Intensified therapy | Standard therapy | OR | P value |
|---|---|---|---|---|
| HScore by therapy | 274 | 199 | P = 0.020 | |
| Treatment escalation per 10-point HScore increase (Firth's logistic regression)Per 10-point HScore increase · univariable | 1.44 | |||
| Treatment escalation per 10-point HScore increase (Firth's logistic regression)Per 10-point HScore increase · adjusted | 1.37 |
Incidence of KD-MAS
- Incidence (22/3786)
- 0.6%
- Coronary involvement
- 77.2%
Treatment pattern
- Stepwise escalation from intravenous immunoglobulin (IVIG) or corticosteroid monotherapy to IVIG plus corticosteroids and, when required, adjunct immunosuppressive/biologic therapy; similar patterns in the literature
Original publication
2016 sJIA-MAS criteria and a step-up therapeutic approach in KD-MAS: insights from a combined cohort and literature review. RMD Open. Published 9 March 2026. doi:10.
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