Autoinflammatory & rare systemic diseases
Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease
Authors' conclusion
IL-1i and IL-6Ri show the highest level of efficacy in the treatment of Still's disease. For MAS, IL-1 and interferon-γ inhibition appear to be effective on a background of high-dose glucocorticoids.
Study context
- Population
- Patients with Still's disease and MAS128 full texts
- Comparison
- Any pharmacological treatment vs any comparator drug or placebo
- Outcome
- Any relevant efficacy and safety event
Methodological detailsinforming the EULAR/PReS guidelines, risk of bias (RoB) assessed with Cochrane RoB and AMSTAR-2, Medline, Embase and Cochrane Library searched, 25 randomised controlled trial (RCTs), 1 CCT, 11 systematic reviews (SRs) published after 2013 and 91 longitudinal observational prospective (LOP)/longitudinal observational retrospective (LOR) studies
- Informing the EULAR/PReS guidelines
- RoB assessed with Cochrane RoB and AMSTAR-2
- Medline, Embase and Cochrane Library searched
- 25 RCTs, 1 CCT, 11 SRs published after 2013 and 91 LOP/LOR studies
Pooled result
As reported in the abstract
ACR50 response rate in Still's disease (meta-analyses of RCTs)
Odds ratio (95% CI)
IL-1i
6.02(2.24–21.36)
IL-6Ri
8.08(1.89–34.57)
Further pooled results
Clinically inactive disease with early IL-1i or IL-6i initiation (retrospective studies)
- Early initiation of IL-1i or IL-6i associated with high rates of clinically inactive disease
Complete response in MAS
- Range of complete response rates
- 53% to 100%
- GCs employed in all patients, often with ciclosporin and/or anakinra
Complete response with emapalumab in MAS (CCT)
- Complete response
- 93%
- Only drug tested in a CCT
Original publication
Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease. Ann Rheum Dis. Published 14 November 2024. doi:10.
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