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CSSs can be genetically determined by inborn errors of immunity (i.e., familial hemophagocytic lymphohistiocytosis) or develop as a complication of infections, chronic inflammatory diseases (e.g., Still disease), or malignancies (e.g., T cell lymphoma). Therapeutic interventions that activate the immune system such as chimeric Ag receptor T cell therapy and immune checkpoint inhibition can also trigger CSSs in the setting of cancer treatment. In this review, the biology of different types of CSSs is explored, and the current knowledge on the involvement of immune pathways and the contribution of host genetics is discussed. The use of animal models to study CSSs is reviewed, and their relevance for human diseases is discussed. Lastly, treatment approaches for CSSs are discussed with a focus on interventions that target immune cells and cytokines.<\/jats:p>","DOI":"10.4049\/jimmunol.2200808","type":"journal-article","created":{"date-parts":[[2023,4,3]],"date-time":"2023-04-03T21:07:43Z","timestamp":1680556063000},"page":"1015-1024","source":"Crossref","is-referenced-by-count":31,"title":["The Multifaceted Immunology of Cytokine Storm Syndrome"],"prefix":"10.1093","volume":"210","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5779-4193","authenticated-orcid":false,"given":"Pui Y","family":"Lee","sequence":"first","affiliation":[{"name":"Division of Immunology, Boston Children\u2019s Hospital, Harvard Medical School , Boston, MA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2661-3086","authenticated-orcid":false,"given":"Randy Q","family":"Cron","sequence":"additional","affiliation":[{"name":"Division of Pediatric Rheumatology, Children\u2019s of Alabama , Birmingham, AL"},{"name":"Department of Pediatrics, University of Alabama Heersink School of Medicine , Birmingham, AL"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2023,4,15]]},"reference":[{"key":"2025021008030036000_r1","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1002\/art.41285","article-title":"On the alert for cytokine storm: immunopathology in COVID-19","volume":"72","author":"Henderson","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"2025021008030036000_r2","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1007\/s40272-019-00367-1","article-title":"Macrophage activation syndrome and secondary hemophagocytic lymphohistiocytosis in childhood inflammatory disorders: diagnosis and management","volume":"22","author":"Henderson","year":"2020","journal-title":"Paediatr. 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