Current treatment in macrophage activation syndrome worldwide: a systematic literature review to inform the METAPHOR project
Articles > Review · 2024
Authors: Francesco Baldo, Remco G A Erkens, Mao Mizuta, Greta Rogani, Federica Lucioni, Claudia Bracaglia, Dirk Foell, Marco Gattorno, Marija Jelusic, Jordi Anton, Paul Brogan, Scott Canna, Shanmuganathan Chandrakasan, Randy Q Cron, Fabrizio De Benedetti, Alexei Grom, Merav Heshin-Bekenstein, AnnaCarin Horne, Raju Khubchandani, Seza Ozen, Pierre Quartier, Angelo Ravelli, Masaki Shimizu, Grant Schulert, Christiaan Scott, Rashmi Sinha, Nicolino Ruperto, Joost F Swart, Sebastiaan Vastert, Francesca Minoia, Kristiina Aalto, Carlos Abud Mendoza, Awatif Abushhaiwia, Constantin Ailioaie, Jonathan Akikusa, Güzide AKSU, Nuray Aktay Ayaz, Ruqaiya Nasser Al Jashmi, Safiya Al-Abrawi, Maria Alessio, Ekaterina Alexeeva, Sulaiman M Al-Mayouf, Abdulaziz AlMutairi, Muatasem Alsuweiti, Rizqi Amalia, Iman Amin, Jordi Anton, Wineke Armbrust, Itziar Astigarraga, Sevcan Bakkaloglu, Reima Bakry, Ozge Basaran, Floricely Basulto May, Jigna Bathia, Cristina Battagliotti, Alexandre Belot, Stefan Berg, Karin Beutel, Blanca Bica, Isabel Bolt, Martin Boyadzhiev, Oksana Boyarchuk, Yaryna Boyko, Claudia Bracaglia, Luciana Breda, Karine Brochard, Paul Brogan, Jurgen Brunner, Segundo Bujan Rivas, Aurelia Calin, Joan Calzada, Marisol Camacho Lovillo, Scott Canna, Elvira Cannizzaro, Roberta Caorsi, Raffaella Carlomagno, Marco Cattalini, Camilla Celani, Kwai Yu Winnie Chan, Sharat Chandra, Shanmuganathan Chandrakasan, Chong U Chang, Vyacheslav Chasnyk, Iryna Chyzheuskaya, Coziana Ciurtin, Daniel Clemente Garulo, Alexis-Virgil Cochino, Alessandro Consolaro, Rita Consolini, Randy Cron, Carlos Henrique M da Silva, Fabrizio De Benedetti, Carmen De Cunto, Arianna De Matteis, Lien De Somer, Fatma Dedeoglu, Chellapandian Deepakbabu, Emanuela Del Giudice, Adriana Soraya Diaz Maldonado, Pavla Dolezalova
Rheumatology, International, Scopus, 2024, vol 64, pp 32-44
DOI: 10.1093/rheumatology/keae391
Summary
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.