{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T17:03:12Z","timestamp":1790960592969,"version":"4.1.0"},"reference-count":47,"publisher":"BMJ","issue":"4","license":[{"start":{"date-parts":[[2023,11,20]],"date-time":"2023-11-20T00:00:00Z","timestamp":1700438400000},"content-version":"unspecified","delay-in-days":19,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["RMD Open"],"accepted":{"date-parts":[[2023,9,12]]},"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Different patient clusters were preliminarily suggested to dissect the clinical heterogeneity in Still\u2019s disease. Thus, we aimed at deriving and validating disease clusters in a multicentre, observational, prospective study to stratify these patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients included in GIRRCS AOSD-study group and AIDA Network Still Disease Registry were assessed if variables for cluster analysis were available (age, systemic score, erythrocyte sedimentation rate (ESR), C reactive protein (CRP) and ferritin). K-means algorithm with Euclidean metric and Elbow plot were used to derive an adequate number of clusters.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>K-means clustering assessment provided four clusters based on means standardised according to z-scores on 349 patients. All clusters mainly presented fever, skin rash and joint involvement. Cluster 1 was composed by 115 patients distinguished by lower values of age and characterised by skin rash myalgia, sore throat and splenomegaly. Cluster 2 included 128 patients identified by lower levels of ESR, ferritin and systemic score; multiorgan manifestations were less frequently observed. Cluster 3 comprised 31 patients categorised by higher levels of CRP and ferritin, they were characterised by fever and joint involvement. Cluster 4 contained 75 patients derived by higher values of age and systemic score. Myalgia, sore throat, liver involvement and life-threatening complications, leading to a high mortality rate, were observed in these patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Four patient clusters in Still\u2019s disease may be recognised by a multidimensional characterisation (\u2018Juvenile\/Transitional\u2019, \u2018Uncomplicated\u2019, \u2018Hyperferritinemic\u2019 and \u2018Catastrophic\u2019). Of interest, cluster 4 was burdened by an increased rate of life-threatening complications and mortality, suggesting a more severe patient group.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1136\/rmdopen-2023-003419","type":"journal-article","created":{"date-parts":[[2023,11,21]],"date-time":"2023-11-21T00:05:15Z","timestamp":1700525115000},"page":"e003419","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":29,"title":["Derivation and validation of four patient clusters in Still\u2019s disease, results from GIRRCS AOSD-study group and AIDA Network Still Disease Registry"],"prefix":"10.1136","volume":"9","clinical-trial-number":[{"clinical-trial-number":"nct05200715","registry":"10.18810\/clinical-trials-gov"},{"clinical-trial-number":"nct05200715","registry":"10.18810\/clinical-trials-gov"}],"author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3487-8551","authenticated-orcid":false,"given":"Piero","family":"Ruscitti","sequence":"first","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Francesco","family":"Masedu","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonio","family":"Vitale","sequence":"additional","affiliation":[{"name":"UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy"},{"name":"Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Beh\u00e7et's Disease Clinic University of Siena, Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilenia","family":"Di Cola","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valeria","family":"Caggiano","sequence":"additional","affiliation":[{"name":"UOC Reumatologia, Azienda Ospedaliero-Universitaria Senese, ERN-RITA Center, Siena, Italy"},{"name":"Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Beh\u00e7et's Disease Clinic University of Siena, Siena, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claudia","family":"Di Muzio","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paola","family":"Cipriani","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marco","family":"Valenti","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Onorina","family":"Berardicurti","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology, Department of Medicine, University of Rome \u201cCampus Biomedico\u201d, School of Medicine, Rome, 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