Journal Article Systematic Review Epublish 10 citations
PubMed: 39317415 DOI: 10.1136/ard-2024-225854
Efficacy and safety of therapies for Still's disease and macrophage activation syndrome (MAS): a systematic review informing the EULAR/PReS guidelines for the management of Still's disease.
Ann Rheum Dis 83(12):1731-1747
Authors: Sara Bindoli, Arianna De Matteis, Stéphane Mitrovic, Bruno Fautrel, Loreto Carmona, Fabrizio De Benedetti
Affiliations
- Rheumatology Unit, Department of Medicine (DIMED), University of Padova, Padova, Italy.
- Division of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, ERN-RITA center, Roma, Italy.
- Department of Rheumatology, Pitié-Salpêtriere Hospital, Assistance Publique-Hôpitaux de Paris, Sorbonne University, Paris, France.
- CRI-IMIDIATE Clinical Research Network and ERN Rita, CEREMAIA Reference Center, Paris, France.
- Pierre Louis Institute of Epidemiology and Public Health, INSERM UMR-S 1136, Paris, France.
- Instituto de Salud Musculoesquelética (INMUSC), Madrid, Spain.
Abstract
OBJECTIVES
To analyse the efficacy and safety of treatments for Still's disease and macrophage activation syndrome (MAS).
METHODS
Medline, Embase and Cochrane Library were searched for clinical trials (randomised, randomised controlled trial (RCT), controlled and clinical controlled trial (CCT)), observational studies (retrospective, longitudinal observational retrospective (LOR), prospective and longitudinal observational prospective (LOP)) and systematic reviews (SRs), in which the populations studied were patients with Still's disease and MAS. The intervention was any pharmacological treatment (approved or under evaluation) versus any comparator drug or placebo, and as outcomes, any relevant efficacy and safety event. The risk of bias (RoB) was assessed with the Cochrane RoB and AMSTAR-2 (Assessing the Methodological Quality of Systematic Reviews-2, version 2) for SRs.
RESULTS
128 full texts were included: 25 RCTs, 1 CCT, 11 SRs published after 2013 and 91 LOP/LOR studies. In Still's disease, interleukin (IL)-1 inhibitors (IL-1i) and IL-6R inhibitors (IL-6i) were the most studied drugs. Two meta-analyses on RCTs showed an OR, to achieve an ARC50 response rate, of 6.02 (95% CI 2.24 to 21.36) and 8.08 (95% CI 1.89 to 34.57) for IL-1i and IL-6Ri, respectively. Retrospective studies showed that early initiation of IL-1i or IL-6i was associated with high rates of clinically inactive disease. In MAS, GCs were employed in all patients, often associated with ciclosporin and/or anakinra. Rates of complete response were reported, with a range from 53% to 100%. Emapalumab was the only drug tested in a CCT, with a complete response of 93%.
CONCLUSION
IL-1i and IL-6Ri show the highest level of efficacy in the treatment of Still's disease. For MAS, IL-1 and interferon-γ inhibition appear to be effective on a background of high-dose glucocorticoids.
MeSH
Macrophage Activation, Safety, Pharmaceutical Preparations, Macrophages, Retrospective Studies, Rheumatic Diseases, Therapeutics, Macrophage Activation Syndrome
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