prikaz prve stranice dokumenta Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
  Download
PDF 1.05 MB
Scientific paper - Original scientific paper
Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
Rheumatology, 64 (2025), 1; 32-44. https://doi.org/10.1093/rheumatology/keae391
(PReS MAS/sJIA Working Party and Paediatric Rheumatology International Trial Organization) Baldo, Francesco; Erkens, Remco G A; Mizuta, Mao; Rogani, Greta; Lucioni, Federica; Bracaglia, Claudia; Foell, Dirk; Gattorno, Marco; Jelušić, Marija; Anton, Jordi; Brogan, Paul; Canna, Scott; Chandrakasan, Shanmuganathan; Cron, Randy Q; De Benedetti, Fabrizio; Grom, Alexei; Heshin-Bekenstein, Merav; Horne, AnnaCarin; Khubchandani, Raju; Ozen, Seza; Quartier, Pierre; Ravelli, Angelo; Shimizu, Masaki; Schulert, Grant; Scott, Christiaan; Sinha, Rashmi; Ruperto, Nicolino; Swart, Joost F; Vastert, Sebastiaan; Minoia, Francesca More authors...

Cite this document

Baldo, F., Erkens, R. G. A., Mizuta, M., Rogani, G., Lucioni, F., Bracaglia, C. ... Minoia, F. (2025). Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project. Rheumatology, 64. (1), 32-44. doi: 10.1093/rheumatology/keae391

Baldo, Francesco, et al. "Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project." Rheumatology, vol. 64, no. 1, 2025, pp. 32-44. https://doi.org/10.1093/rheumatology/keae391

Baldo, Francesco, Remco G A Erkens, Mao Mizuta, Greta Rogani, Federica Lucioni, Claudia Bracaglia, Dirk Foell, et al. "Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project." Rheumatology 64, no. 1 (2025): 32-44. https://doi.org/10.1093/rheumatology/keae391

Baldo, F., et al. (2025) 'Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project', Rheumatology, 64(1), pp. 32-44. doi: 10.1093/rheumatology/keae391

Baldo F, Erkens RGA, Mizuta M, Rogani G, Lucioni F, Bracaglia C, and sur.. Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project. Rheumatology [Internet]. 2025 January 01 [cited 2025 April 04];64(1):32-44. doi: 10.1093/rheumatology/keae391

F. Baldo, et al., "Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project", Rheumatology, vol. 64, no. 1, pp. 32-44, January 2025. [Online]. Available at: https://urn.nsk.hr/urn:nbn:hr:105:267685. [Accessed: 04 April 2025]

Please login to the repository to save this object to your list.
Metadata
Title (english)Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
AuthorFrancesco Baldo
AuthorRemco G A Erkens
AuthorMao Mizuta
AuthorGreta Rogani
AuthorFederica Lucioni
AuthorClaudia Bracaglia
AuthorDirk Foell
AuthorMarco Gattorno
AuthorMarija Jelušić
AuthorJordi Anton
AuthorPaul Brogan
AuthorScott Canna
AuthorShanmuganathan Chandrakasan
AuthorRandy Q Cron
AuthorFabrizio De Benedetti
AuthorAlexei Grom
AuthorMerav Heshin-Bekenstein
AuthorAnnaCarin Horne
AuthorRaju Khubchandani
AuthorSeza Ozen
AuthorPierre Quartier
AuthorAngelo Ravelli
AuthorMasaki Shimizu
AuthorGrant Schulert
AuthorChristiaan Scott
AuthorRashmi Sinha
AuthorNicolino Ruperto
AuthorJoost F Swart
AuthorSebastiaan Vastert
AuthorFrancesca Minoia
CollaborationPReS MAS/sJIA Working Party and Paediatric Rheumatology International Trial Organization
Author's institutionUniversity of Zagreb
School of Medicine
(Department of Pediatrics)
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Pediatrics
Abstract (english)
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%); dexamethasone was used in 7% of patients. Ciclosporin was reported in a wide range of patients according to different cohorts. Anakinra was used in 179 MAS patients, with a favourable outcome in 83% of sJIA-MAS. Etoposide was described by 11 studies, mainly as part of HLH-94/04 protocol. Emapalumab was the only medication tested in a clinical trial in 14 sJIA-MAS, with 93% of MAS remission. Ruxolitinib was the most reported Janus kinase inhibitor in MAS. Conclusion: High-dose GCs together with IL-1 and IFNγ inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS. However, the global level of evidence on MAS treatment, especially in other conditions, is still poor and requires standardized studies to be confirmed.
Keywords (english)
Languageenglish
Publication typeScientific paper - Original scientific paper
Publication statusPublished
Peer reviewPeer review
Publication versionPublished version
Journal titleRheumatology
Numberingvol. 64, no. 1, pp. 32-44
p-ISSN1462-0324
e-ISSN1462-0332
DOIhttps://doi.org/10.1093/rheumatology/keae391
URN:NBNurn:nbn:hr:105:267685
Publication2025-01-01
Document URLhttps://academic.oup.com/rheumatology/article/64/1/32/7721438
Type of resourceText
Access conditionsOpen access
Terms of useLicence Attribution 4.0 International (CC BY 4.0) icon
Created on2025-04-04 08:21:58