prikaz prve stranice dokumenta Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
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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 Više autora...

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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, br. 1, 2025, str. 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, br. 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), str. 32-44. doi: 10.1093/rheumatology/keae391

Baldo F, Erkens RGA, Mizuta M, Rogani G, Lucioni F, Bracaglia C, i sur.. Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project. Rheumatology [Internet]. 01.01.2025. [pristupljeno 04.04.2025.];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, br. 1, str. 32-44, Siječanj 2025. [Online]. Dostupno na: https://urn.nsk.hr/urn:nbn:hr:105:267685. [Citirano: 04.04.2025.]

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Naslov (engleski)Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
AutorFrancesco Baldo
AutorRemco G A Erkens
AutorMao Mizuta
AutorGreta Rogani
AutorFederica Lucioni
AutorClaudia Bracaglia
AutorDirk Foell
AutorMarco Gattorno
AutorMarija Jelušić
AutorJordi Anton
AutorPaul Brogan
AutorScott Canna
AutorShanmuganathan Chandrakasan
AutorRandy Q Cron
AutorFabrizio De Benedetti
AutorAlexei Grom
AutorMerav Heshin-Bekenstein
AutorAnnaCarin Horne
AutorRaju Khubchandani
AutorSeza Ozen
AutorPierre Quartier
AutorAngelo Ravelli
AutorMasaki Shimizu
AutorGrant Schulert
AutorChristiaan Scott
AutorRashmi Sinha
AutorNicolino Ruperto
AutorJoost F Swart
AutorSebastiaan Vastert
AutorFrancesca Minoia
KolaboracijaPReS MAS/sJIA Working Party and Paediatric Rheumatology International Trial Organization
Autorova ustanovaSveučilište u Zagrebu
Medicinski fakultet
(KATEDRA ZA PEDIJATRIJU)
Znanstveno / umjetničko
područje, polje i grana		BIOMEDICINA I ZDRAVSTVO
Kliničke medicinske znanosti
Pedijatrija
Sažetak (engleski)
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.
Ključne riječi (engleski)
Jezikengleski
Vrsta publikacijeZnanstveni rad - Izvorni znanstveni rad
Status objaveObjavljen
Vrsta recenzijeRecenziran
Verzija publikacijeObjavljena verzija rada (izdavačev PDF)
Naslov časopisaRheumatology
Brojčani podacivol. 64, br. 1, str. 32-44
p-ISSN1462-0324
e-ISSN1462-0332
DOIhttps://doi.org/10.1093/rheumatology/keae391
URN:NBNurn:nbn:hr:105:267685
Datum objave publikacije2025-01-01
URL dokumentahttps://academic.oup.com/rheumatology/article/64/1/32/7721438
Vrsta resursaTekst
Prava pristupaOtvoreni pristup
Uvjeti korištenjaIkona licence Imenovanje 4.0 međunarodna (CC BY 4.0)
Datum i vrijeme pohrane2025-04-04 08:21:58