{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T00:21:41Z","timestamp":1791246101968,"version":"4.1.0"},"reference-count":145,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T00:00:00Z","timestamp":1714089600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Macrophage activation syndrome (MAS) is a rare complication of autoimmune inflammatory rheumatic diseases (AIIRD) characterized by a progressive and life-threatening condition with features including cytokine storm and hemophagocytosis. Predisposing factors are typically associated with microbial infections, genetic factors (distinct from typical genetically related hemophagocytic lymphohistiocytosis (HLH)), and inappropriate immune system overactivation. Clinical features include unremitting fever, generalized rash, hepatosplenomegaly, lymphadenopathy, anemia, worsening liver function, and neurological involvement. MAS can occur in various AIIRDs, including but not limited to systemic juvenile idiopathic arthritis (sJIA), adult-onset Still\u2019s disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease (KD), juvenile dermatomyositis (JDM), rheumatoid arthritis (RA), and Sj\u00f6gren\u2019s syndrome (SS), etc. Although progress has been made in understanding the pathogenesis and treatment of MAS, it is important to recognize the differences between different diseases and the various treatment options available. This article summarizes the cell types and cytokines involved in MAS-related diseases, the heterogeneity, and treatment options, while also comparing it to genetically related HLH.<\/jats:p>","DOI":"10.3389\/fimmu.2024.1389710","type":"journal-article","created":{"date-parts":[[2024,4,26]],"date-time":"2024-04-26T05:01:32Z","timestamp":1714107692000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":31,"title":["Heterogeneity of macrophage activation syndrome and treatment progression"],"prefix":"10.3389","volume":"15","author":[{"given":"Yuanji","family":"Dong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huaxiang","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2024,4,26]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"e27929","DOI":"10.1002\/pbc.27929","article-title":"Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)","volume":"66","author":"Jordan","year":"2019","journal-title":"Pediatr Blood Cancer"},{"key":"B2","doi-asserted-by":"publisher","first-page":"5","DOI":"10.1093\/rheumatology\/key006","article-title":"Macrophage activation syndrome in adults: recent advances in pathophysiology, diagnosis and treatment","volume":"58","author":"Carter","year":"2019","journal-title":"Rheumatol (Oxford)"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2019000936","article-title":"Pediatric hemophagocytic lymphohistiocytosis","volume":"135","author":"Canna","year":"2020","journal-title":"Blood"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1136\/ard-2023-224123","article-title":"The 2022 EULAR\/ACR points to consider at the early stages of diagnosis and management of suspected hemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS)","volume":"82","author":"Shakoory","year":"2023","journal-title":"Ann Rheum Dis"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa299","article-title":"Comparison of serum cytokine profiles in macrophage activation syndrome complicating different background rheumatic diseases in children","volume":"60","author":"Mizuta","year":"2021","journal-title":"Rheumatol (Oxford)"},{"key":"B6","doi-asserted-by":"publisher","first-page":"198","DOI":"10.1016\/j.semarthrit.2020.12.007","article-title":"Understanding of cytokines and targeted therapy in macrophage activation syndrome","volume":"51","author":"Tang","year":"2021","journal-title":"Semin Arthritis Rheum"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1038\/s41435-020-0098-4","article-title":"The genetics of macrophage activation syndrome","volume":"21","author":"Schulert","year":"2020","journal-title":"Genes Immun"},{"key":"B8","article-title":"Haemophagocytic syndrome in rheumatic patients. 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