{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T06:00:37Z","timestamp":1785477637797,"version":"3.56.0"},"reference-count":31,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T00:00:00Z","timestamp":1785456000000},"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":"\n Background<\/jats:title>\n Hemophagocytic lymphohistiocytosis\/macrophage activation syndrome (HLH\/MAS) is one of the most common complications of adult-onset Still\u2019s disease (AOSD), and a leading cause of organ failure and mortality in these patients. Early diagnosis and management are crucial but can be challenging. Our study aims to develop a tool for predicting the risk of HLH\/MAS in AOSD patients.<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n The study recruited AOSD patients admitted to Ruijin Hospital from May 2018 to May 2024. Clinical features and laboratory test results were collected within the first 3 days of hospitalization. Predictor variables were selected using LASSO regression, and a logistic regression model was subsequently developed to predict HLH\/MAS. Model performance was assessed through calibration curves, receiver operating characteristic (ROC) curve, decision curve analysis (DCA), and bootstrap internal validation.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n This retrospective cohort study included 190 patients with AOSD. Those who developed HLH\/MAS had a higher incidence of fever and splenomegaly at admission. They also exhibited more significant reductions in blood cell counts, elevated inflammatory markers, liver dysfunction, and coagulopathy compared to patients without HLH\/MAS. We developed the HLH\/MAS Risk Score, which included five key predictors\u2014body temperature, platelet count, D-dimer levels, systemic score, and initial glucocorticoid response\u2014to assess the HLH\/MAS risk in hospitalized AOSD patients. This model was visualized and demonstrated robust predictive performance, achieving an AUC of 0.86. Comparative analysis with other MAS-related models confirmed that the HLH\/MAS Risk Score had competitive discriminative power.<\/jats:p>\n <\/jats:sec>\n \n Conclusions<\/jats:title>\n In this study, we developed the HLH\/MAS Risk Score, a predictive tool designed to assess the risk of HLH\/MAS in AOSD patients during hospitalization. The model demonstrated strong predictive performance and provides a reliable, easy-to-use tool for the early identification of patients at high risk for HLH\/MAS. Further external validation is needed to confirm its clinical applicability across diverse populations.<\/jats:p>\n <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1709136","type":"journal-article","created":{"date-parts":[[2026,7,31]],"date-time":"2026-07-31T05:37:55Z","timestamp":1785476275000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Development of a predictive tool for hemophagocytic lymphohistiocytosis\/macrophage activation syndrome risk in adult-onset Still\u2019s disease"],"prefix":"10.3389","volume":"17","author":[{"given":"Xinyue","family":"Hong","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Linzi","family":"Meng","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ningqi","family":"Dai","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hongxia","family":"Shi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Traditional Chinese Medicine Hospital of Xinjiang Uygur Autonomous Region","place":["Urumqi, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yutong","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiongyi","family":"Hu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yue","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Honglei","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jianfen","family":"Meng","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jinchao","family":"Jia","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mengyan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chengde","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yaping","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jialin","family":"Teng","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huihui","family":"Chi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine","place":["Shanghai, 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