Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy
Authors' conclusion
Anti-IL-1/-6 therapies can mask the clinical and laboratory features of MAS, and proposed guidelines for MAS classification criteria may not be met.
Study context
- Population
- Patients with systemic juvenile idiopathic arthritis (sJIA)-associated macrophage activation syndrome (MAS) followed up at sixteen paediatric rheumatology centres across the country162 patients
- Participants
- 45 vs 155
- Exposure
- MAS events on anti-IL-1/−6 biologics vs MAS events without biological treatment in the last three months
- Anti-IL-1/−6 biologicsn = 45
- No biologicsn = 155reference
- Outcome
- Clinical and laboratory features of MAS on biologics vs without
Methodological detailsmulticentre (sixteen paediatric rheumatology centres)
- Multicentre (sixteen paediatric rheumatology centres)
Main finding
As reported in the abstract
Clinical and laboratory features of MAS (lower in Anti-IL-1/−6 biologics)
- Platelet count, 109/l: 128 (72-232) vs 199 (130-371)
- Ferritin on admission, ng/ml: 1107 (676-2050) vs 2863 (1193-9562)
- CRP, mg/l: 15.4 (2.9-56) vs 90 (32-160)
- ESR, mm/h: 13 (3-36) vs 43.5 (13-77)
- Fever duration, days: 5 (4-7.5) vs 10 (7-14.3)
- All lower in the group under anti-IL-1/−6 biologics
Observational data: associations, not effects.
Further findings
Not meeting the 2016 MAS classification criteria at presentation, among patients treated with biologics
- Patients on biologics not meeting 2016 MAS classification criteria at presentation
- 26.6%
Hepatomegaly and splenomegaly by biologic
- Rates of hepatomegaly and splenomegaly relatively lower in the canakinumab-treated group than with other biologicals or no biologicals
Original publication
Macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis on anti-interleukin-1 or -6 therapy. Rheumatology (Oxford). Published 5 March 2024. doi:10.
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