{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,22]],"date-time":"2026-09-22T19:13:22Z","timestamp":1790104402969,"version":"4.0.1"},"reference-count":145,"publisher":"MDPI AG","issue":"4","license":[{"start":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T00:00:00Z","timestamp":1613088000000},"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>Adult-onset Still\u2019s disease (AoSD) is a rare systemic autoinflammatory disease characterized by arthritis, spiking fever, skin rash and elevated ferritin levels. The reason behind the nomenclature of this condition is that AoSD shares certain symptoms with Still\u2019s disease in children, currently named systemic-onset juvenile idiopathic arthritis. Immune dysregulation plays a central role in AoSD and is characterized by pathogenic involvement of both arms of the immune system. Furthermore, the past two decades have seen a large body of immunological research on cytokines, which has attributed to both a better understanding of AoSD and revolutionary advances in treatment. Additionally, recent studies have introduced a new approach by grouping patients with AoSD into only two phenotypes: one with predominantly systemic features and one with a chronic articular disease course. Diagnosis presupposes an extensive diagnostic workup to rule out infections and malignancies. The severe end of the spectrum of this disease is secondary haemophagocytic lymphohistiocytosis, better known as macrophage activation syndrome. In this review, we discuss current research conducted on the pathogenesis, diagnosis, classification, biomarkers and complications of AoSD, as well as the treatment strategy at each stage of the disease course. We also highlight the similarities and differences between AoSD and systemic-onset juvenile idiopathic arthritis. There is a considerable need for large multicentric prospective trials.<\/jats:p>","DOI":"10.3390\/jcm10040733","type":"journal-article","created":{"date-parts":[[2021,2,12]],"date-time":"2021-02-12T18:45:00Z","timestamp":1613155500000},"page":"733","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":78,"title":["Adult-Onset Still\u2019s Disease: Clinical Aspects and Therapeutic Approach"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5923-2557","authenticated-orcid":false,"given":"Stylianos","family":"Tomaras","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Helios Clinic Vogelsang-Gommern, 39245 Gommern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3407-7631","authenticated-orcid":false,"given":"Carl Christoph","family":"Goetzke","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonology, Immunology and Critical Care Medicine, Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin, Humboldt-Universit\u00e4t zu Berlin, and Berlin Institute of Health (BIH), 10117 Berlin, Germany"},{"name":"German Rheumatism Research Center (DRFZ), Leibniz Association, 10117 Berlin, Germany"},{"name":"Berlin Institute of Health, 10178 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tilmann","family":"Kallinich","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Division of Pulmonology, Immunology and Critical Care Medicine, Charit\u00e9\u2013Universit\u00e4tsmedizin Berlin, Corporate Member of Freie Universit\u00e4t Berlin, Humboldt-Universit\u00e4t zu Berlin, and Berlin Institute of Health (BIH), 10117 Berlin, Germany"},{"name":"German Rheumatism Research Center (DRFZ), Leibniz Association, 10117 Berlin, Germany"},{"name":"Berlin Institute of Health, 10178 Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9872-5282","authenticated-orcid":false,"given":"Eugen","family":"Feist","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Helios Clinic Vogelsang-Gommern, 39245 Gommern, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,12]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1016\/j.berh.2008.08.006","article-title":"Adult-onset Still disease","volume":"22","author":"Fautrel","year":"2008","journal-title":"Best Pract. 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