Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project.

ABSTRACT:

Objective

To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice.

Methods

A systematic literature search was performed in both EMBASE and PubMed databases. Paper screening was done by two independent teams based on agreed criteria. Data extraction was standardized following the PICO framework. A panel of experts assessed paper validity, using the Joanna Briggs Institute appraisal tools and category of evidence (CoE) according to EULAR procedure.

Results

Fifty-seven papers were finally included (80% retrospective case-series), describing 1148 patients with MAS: 889 systemic juvenile idiopathic arthritis (sJIA), 137 systemic lupus erythematosus (SLE), 69 Kawasaki disease (KD) and 53 oth

SUBMITTER: Baldo F 

PROVIDER: S-EPMC11701305 | biostudies-literature | 2025 Jan

REPOSITORIES: biostudies-literature