Pediatric rheumatology

Efficacy and safety of emapalumab in macrophage activation syndrome

Ann Rheum DisPublished Prospective single-arm trial

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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