Treatment approaches to macrophage activation syndrome in real life and priorities for future research: results from the international METAPHOR project.
Обработка статьи · этапы, ошибки и управление
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Baldo Francesco, Rogani Greta, Erkens Remco G A, Mizuta Mao, 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, PReS MAS/sJIA Working Party and the Paediatric Rheumatology International Trial Organisation (PRINTO)
Аннотация
METHODS: An international web-based survey was developed by a multidisciplinary expert panel together with a patient representative, following a systematic literature review. The survey was anonymously completed through PReS/PRINTO and the Histiocyte Society networks (October-December 2023). Treatment strategies for MAS across different rheumatologic contexts were explored. Responses were analyzed descriptively and stratified by geography, subspecialty, clinical experience, and underlying disease.
RESULTS: A total of 287 physicians from 64 countries completed the survey, the majority being pediatric rheumatologists with more than 5 years of experience. Glucocorticoids, particularly intravenous methylprednisolone, were consistently identified as 1st-line therapy across all MAS subtypes (for 44% of clinicians as monotherapy). Anakinra, ciclosporin, and intravenous immunoglobulins were the most frequently combined agents. However, their use varied markedly by underlying disease and geographical region, with anakinra preferentially used in SD-MAS. Striking global disparities in drug availability, particularly for anakinra and emapalumab, significantly influenced treatment choices and were associated with increased reliance on steroid monotherapy.
CONCLUSION: MAS management remains highly heterogeneous worldwide. While glucocorticoids remain foundational, reducing steroid reliance through equitable access to immunomodulatory therapies and advancing mechanism-based strategies represent critical priorities in optimizing MAS care.