{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T16:19:36Z","timestamp":1791908376861,"version":"4.3.4"},"reference-count":71,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2021,12,31]],"date-time":"2021-12-31T00:00:00Z","timestamp":1640908800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Distinguishing between macrophage activation syndrome (MAS) and a simple flare of Still\u2019s disease (SD) may be challenging. We sought to clarify the clinical features and outcome of MAS in SD and to explore predictive factors of MAS development. Demographic and clinical data, treatments, and outcomes were recorded in a cohort of 206 SD patients. SD patients with and without MAS were compared. To explore predictive factors for the development of MAS, patients were compared at the time of SD diagnosis. Twenty (9.7%) patients experienced MAS, which was inaugural in 12 cases. Patients with MAS were more likely to have hepatomegaly (OR, 3.71; 95% CI, 1.14\u201311.2; p = 0.03) and neurological symptoms (OR, 4.43; 95% CI, 1.08\u201315.3; p = 0.04) than patients without MAS. Cytopenias, abnormal liver tests, and coagulation disorders were significantly more frequent in patients with MAS; lactate dehydrogenase and serum ferritin levels were significantly higher. An optimized threshold of 3500 \u03bcg\/L for serum ferritin yielded a sensitivity (Se) of 85% and a negative predictive value (NPV) of 97% for identifying patients with\/without MAS. Survival analysis showed that a high ferritin level at the time of SD diagnosis was predictive of MAS development (p &lt; 0.001). Specific factors, including neurological symptoms, cytopenias, elevated LDH, and coagulopathy, may contribute to the early detection of MAS. Extreme hyperferritinemia at the onset of SD is a prognostic factor for the development of MAS.<\/jats:p>","DOI":"10.3390\/jcm11010206","type":"journal-article","created":{"date-parts":[[2022,1,3]],"date-time":"2022-01-03T20:49:38Z","timestamp":1641242978000},"page":"206","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Detection and Prediction of Macrophage Activation Syndrome in Still\u2019s Disease"],"prefix":"10.3390","volume":"11","author":[{"given":"Cl\u00e9ment","family":"Javaux","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Thomas","family":"El-Jammal","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierre-Antoine","family":"Neau","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6496-4496","authenticated-orcid":false,"given":"Nicolas","family":"Fournier","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4245-8039","authenticated-orcid":false,"given":"Mathieu","family":"Gerfaud-Valentin","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laurent","family":"Perard","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Saint Luc Saint Joseph Hospital, 69007 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marine","family":"Fouillet-Desjonqueres","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nephrology, Rheumatology, Dermatology, M\u00e8re-Enfant Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69500 Bron, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie","family":"Le Scanff","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Villefranche-sur-Sa\u00f4ne Hospital, 69400 Gleize, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emmanuelle","family":"Vignot","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Edouard Herriot University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Durupt","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Lyon-Sud University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69310 Pierre-Benite, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Arnaud","family":"Hot","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Edouard Herriot University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4902-5332","authenticated-orcid":false,"given":"Alexandre","family":"Belot","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nephrology, Rheumatology, Dermatology, M\u00e8re-Enfant Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69500 Bron, France"},{"name":"CIRI (Centre International de Recherche en Infectiologie), Inserm U1111, CNRS, UMR5308, ENS de Lyon, Universit\u00e9 Claude Bernard Lyon 1, 69007 Lyon, France"},{"name":"Lyon Immunopathology Federation (LIFE), 69000 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Isabelle","family":"Durieu","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Lyon-Sud University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69310 Pierre-Benite, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0687-8565","authenticated-orcid":false,"given":"Thomas","family":"Henry","sequence":"additional","affiliation":[{"name":"CIRI (Centre International de Recherche en Infectiologie), Inserm U1111, CNRS, UMR5308, ENS de Lyon, Universit\u00e9 Claude Bernard Lyon 1, 69007 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3070-3842","authenticated-orcid":false,"given":"Pascal","family":"S\u00e8ve","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"},{"name":"Research on Healthcare Performance (RESHAPE), INSERM U1290, Universit\u00e9 Claude Bernard Lyon 1, 69003 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5249-3650","authenticated-orcid":false,"given":"Yvan","family":"Jamilloux","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Universit\u00e9 Claude Bernard-Lyon 1, 69004 Lyon, France"},{"name":"CIRI (Centre International de Recherche en Infectiologie), Inserm U1111, CNRS, UMR5308, ENS de Lyon, Universit\u00e9 Claude Bernard Lyon 1, 69007 Lyon, France"},{"name":"Lyon Immunopathology Federation (LIFE), 69000 Lyon, France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,12,31]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1136\/ard.30.2.121","article-title":"Still\u2019s disease in the adult","volume":"30","author":"Bywaters","year":"1971","journal-title":"Ann. 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