Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
Authors' conclusion
High-dose GCs together with IL-1 and IFNγ inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS.
Study context
- Population
- Patients with macrophage activation syndrome (MAS) worldwide: Systemic juvenile idiopathic arthritis (sJIA), systemic lupus erythematosus (SLE), Kawasaki disease and other rheumatological conditions57 papers
- Participants
- 1,148 patients
- Outcome
- Current treatment of MAS
Methodological detailsEMBASE and PubMed, two independent screening teams, PICO framework data extraction, Joanna Briggs Institute appraisal tools, EULAR category of evidence
- EMBASE and PubMed
- Two independent screening teams
- PICO framework data extraction
- Joanna Briggs Institute appraisal tools
- EULAR category of evidence
Main finding
As reported in the abstract
- Papers mentioning glucocorticoids (GCs) (all), mostly methylprednisolone and prednisolone
- 90%
- Patients receiving dexamethasone
- 7%
- MAS patients treated with anakinra
- 179
- Favourable outcome with anakinra in sJIA-MAS
- 83%
- Studies describing etoposide, mainly as part of HLH-94/04 protocol
- 11
- MAS remission with emapalumab (only medication tested in a clinical trial; 14 sJIA-MAS)
- 93%
Original publication
Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project. Rheumatology (Oxford). Published 26 July 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →