Efficacy and safety of emapalumab in macrophage activation syndrome
Authors' conclusion
Neutralisation of IFNγ with emapalumab was efficacious in inducing remission of MAS secondary to sJIA or AOSD in patients who had failed high-dose glucocorticoids.
Study context
- Population
- Patients with macrophage activation syndrome (MAS) secondary to systemic juvenile idiopathic arthritis (sJIA) or adult-onset Still's disease (AOSD) who had failed high-dose GC, with or without anakinra and/or ciclosporinall completed the trial
- Participants
- 14 patients
- Outcome
- MAS remission, based on clinical and laboratory criteria
- Follow-up
- Week 8
Methodological detailslong-term (12 months) follow-up study
- Long-term (12 months) follow-up study
Main finding
As reported in the abstract
MAS remission
MAS remission achieved by week 8
13 of the 14 patients
Median time to MAS remission
25 days
Observational data: associations, not effects.
Further findings
IFNγ neutralisation (serum CXCL9)
- Rapid neutralisation of IFNγ activity, shown by a prompt decrease in serum CXCL9 levels
Survival · end of follow-up
- All patients alive at the end of follow-up
Original publication
Efficacy and safety of emapalumab in macrophage activation syndrome. Ann Rheum Dis. Published 31 March 2023. doi:10. 1136/ ard-2022-223739
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