{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,9]],"date-time":"2026-09-09T15:59:00Z","timestamp":1788969540873,"version":"build-2803163510"},"reference-count":39,"publisher":"MDPI AG","issue":"22","license":[{"start":{"date-parts":[[2022,11,12]],"date-time":"2022-11-12T00:00:00Z","timestamp":1668211200000},"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>Still\u2019s disease (SD) is a heterogeneous autoinflammatory disorder for which several phenotypes have been described. We conducted a retrospective study to re-evaluate the dichotomous view of the disease, to compare the juvenile and adult forms, and to look for prognostic factors. We collected data from ten French centers, seeking patients with a diagnosis of adult-onset SD (AOSD) or systemic juvenile idiopathic arthritis (sJIA). We identified 238 patients, 152 (64%) of whom had AOSD while 86 (36%) had sJIA. The median age at SD onset was 26.6 years. In patients with identifiable patterns, the course of SD was systemic in 159 patients (74%), chronic in 55 (26%). Sore throat and myalgia were more frequent in patients with AOSD. Abnormal liver tests, serum ferritin and C-reactive protein levels were higher in AOSD group. Fever and skin rash were predictive of complete remission or recovery and high lactate dehydrogenase level was a poor prognosis factor. Symptoms such as splenomegaly, skin rash, high polymorphonuclear neutrophils count and macrophage activation syndrome were predictive of a systemic phenotype. Overall, there were no major differences between sJIA and AOSD. Our results are consistent with the \u201cbiphasic\u201d model of an autoinflammatory disease that can progress to chronic arthritis if not treated early.<\/jats:p>","DOI":"10.3390\/jcm11226703","type":"journal-article","created":{"date-parts":[[2022,11,14]],"date-time":"2022-11-14T04:24:10Z","timestamp":1668399850000},"page":"6703","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":14,"title":["The Spectrum of Still\u2019s Disease: A Comparative Analysis of Phenotypic Forms in a Cohort of 238 Patients"],"prefix":"10.3390","volume":"11","author":[{"given":"Pierre-Antoine","family":"Neau","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":"Cl\u00e9ment","family":"Javaux","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\/0000-0002-0209-8692","authenticated-orcid":false,"given":"Orlane","family":"Chol","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Grenoble Alpes University Hospital, 38043 Grenoble, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L\u00e9opold","family":"Adela\u00efde","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Vienne-Lucien HUSSEL Hospital, 38200 Vienne, France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8511-903X","authenticated-orcid":false,"given":"Kim","family":"Ly","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Limoges University Hospital, 87042 Limoges, 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":"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\/0000-0003-3070-3842","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":[[2022,11,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1080\/1744666X.2018.1533403","article-title":"Pathogenesis of Adult Onset Still\u2019s Disease: Current Understanding and New Insights","volume":"14","author":"Ruscitti","year":"2018","journal-title":"Expert Rev. 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