ReviewRMD open2026
2016 sJIA-MAS criteria and a step-up therapeutic approach in KD-MAS: insights from a combined cohort and literature review.
Review in RMD open, 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.
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8 authors.
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Abstract
objectivesTo describe diagnostic framework classification, treatment patterns and outcomes in Kawasaki disease-associated macrophage activation syndrome (KD-MAS) using a single-centre cohort and a structured descriptive synthesis of published cases to inform hypothesis generation and future refinement of diagnostic and management strategies for KD-MAS.
methodsWe performed a retrospective single-centre cohort study. MAS was classified by haemophagocytic lymphohistiocytosis (HLH)-2004, HLH-2009 or 2016 systemic juvenile idiopathic arthritis (sJIA)-MAS criteria. Data were abstracted around a prespecified MAS window; severity was indexed by haemophagocytic syndrome diagnostic score (HScore) and the association between HScore and treatment escalation was assessed using Firth's logistic regression. In parallel, we conducted a literature review.
resultsIn our centre, incidence was 0.6% (22/3786); mean age was 3.72 years; coronary involvement was 77.2%. The proportions of clinician-diagnosed KD-MAS cases fulfilling each framework were HLH-2004 14/22, HLH-2009 18/22 and 2016 sJIA-MAS 20/22; 11 met all three. Cytopenias, liver dysfunction and coagulopathy were frequent; management followed stepwise escalation from intravenous immunoglobulin (IVIG) or corticosteroid monotherapy to IVIG plus corticosteroids and, when required, to adjunct immunosuppressive/biologic therapy; similar patterns appeared in the literature. HScore was higher with intensified therapy than with standard therapy (median 274 vs 199; p=0.020). Each 10-point HScore increase was associated with higher odds of escalation (OR 1.44 univariable; 1.37 adjusted).
conclusionThe 2016 sJIA-MAS criteria demonstrate the highest proportion fulfilling criteria in our cohort for KD-MAS. Treatment in our cohort and in published cases generally reflected a stepwise, typically progressing from IVIG±glucocorticoids to adjunct immunosuppressants/biologics. Higher HScores co-occurred with escalation, suggesting they capture baseline severity; prospective multicentre studies are needed to test incremental value and risk-stratification thresholds.
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