Current Treatment in Macrophage Activation Syndrome Worldwide
Author Information
Author(s): Baldo Francesco, Erkens Remco G A, Mizuta Mao, Rogani Greta, Lucioni Federica, Bracaglia Claudia, Foell Dirk, Gattorno Marco, Jelusic 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
Primary Institution: Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan, Italy
Hypothesis
To assess current treatment in macrophage activation syndrome (MAS) worldwide and to highlight any areas of major heterogeneity of practice.
Conclusion
High-dose glucocorticoids together with IL-1 and IFNγ inhibitors have shown efficacy in MAS, especially in sJIA-associated MAS, but the global level of evidence on MAS treatment is still poor.
Supporting Evidence
- High-dose glucocorticoids were used in 90% of patients.
- Anakinra showed a complete response in 83% of sJIA-MAS patients.
- Emapalumab led to a 93% remission rate in a clinical trial for sJIA-MAS.
Takeaway
Doctors are trying to find the best ways to treat a serious condition called macrophage activation syndrome, which can happen with other illnesses. They found that some medicines work well, but there are still many questions about the best treatments.
Methodology
A systematic literature search was performed in EMBASE and PubMed, with data extraction standardized following the PICO framework.
Potential Biases
Most papers had low or moderate validity, and almost all were classified with a CoE of 3 or 4.
Limitations
The global level of evidence on MAS treatment is still poor, with high biases and scarce reliability in attributing efficacy to specific medications due to the retrospective nature and heterogeneity of most studies.
Participant Demographics
The study included 1148 patients with MAS, primarily from systemic juvenile idiopathic arthritis (sJIA), systemic lupus erythematosus (SLE), and Kawasaki disease (KD).
Digital Object Identifier (DOI)
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