Year: 2026
Type: article
Study design: Systematic Review
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 other rheumatological conditions. Fourteen and 11 studies specified data on MAS associated to SLE and KD, respectively. All papers mentioned glucocorticoids (GCs), mostly methylprednisolone and prednisolone (90%)
Institutions: Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Istituto Ortopedico Gaetano Pini, University Medical Center Utrecht, Kobe Children's Hospital, Bambino Gesù Children's Hospital, Istituti di Ricovero e Cura a Carattere Scientifico, University Hospital Münster, Istituto Giannina Gaslini, University Hospital Centre Zagreb
Language: English
Subfield: Hematology
Field: Medicine
Domain: Health Sciences
Open Access status: green
OpenAlex ID: W7155453214
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