{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T16:07:30Z","timestamp":1772726850294,"version":"3.50.1"},"reference-count":4,"publisher":"SMC Media","issue":"3","license":[{"start":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T00:00:00Z","timestamp":1771200000000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Introduction:\u00a0Adult-onset Still\u2019s disease (AOSD) is a rare systemic autoinflammatory disorder characterized by dysregulation of the innate immune system and excessive cytokine production. Its presentation is often non-specific and heterogeneous, making diagnosis challenging and one of exclusion. Case description:\u00a0We report the case of an 18-year-old woman admitted with quotidian fever, frequent episodes of tonsillitis, intermittent skin rash and progressive distal lower limb weakness. Neurological assessment demonstrated progressive motor neuropathy. Laboratory findings revealed neutrophilic leucocytosis, significantly elevated inflammatory markers and hyperferritinaemia. Extensive investigation excluded infectious, malignant and autoimmune causes, and the patient met the Yamaguchi classification criteria for AOSD. Treatment with systemic corticosteroids, colchicine and the interleukin-1 receptor antagonist resulted in rapid and sustained clinical improvement. Conclusion:\u00a0This case highlights the diagnostic complexity of AOSD and emphasizes the importance of considering this diagnosis in patients with persistent fever, hyperferritinaemia and rare neurological manifestations.<\/jats:p>","DOI":"10.12890\/2026_006231","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T15:21:13Z","timestamp":1771255273000},"source":"Crossref","is-referenced-by-count":0,"title":["Adult-onset Still\u2019s disease presenting with progressive motor neuropathy"],"prefix":"10.12890","volume":"13","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5420-803X","authenticated-orcid":false,"given":"Ana Rita","family":"Freire","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Unidade Local de Sa\u00fade da Regi\u00e3o de Leiria \u2013 Hospital de Santo Andr\u00e9, Leiria, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-1684-1779","authenticated-orcid":false,"given":"N\u00e1dia","family":"Santos","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Unidade Local de Sa\u00fade da Regi\u00e3o de Leiria \u2013 Hospital de Santo Andr\u00e9, Leiria, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-8157-0434","authenticated-orcid":false,"given":"Patr\u00edcia","family":"Fernandes","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Unidade Local de Sa\u00fade da Regi\u00e3o de Leiria \u2013 Hospital de Santo Andr\u00e9, Leiria, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5314-410X","authenticated-orcid":false,"given":"Diana","family":"Moura","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Unidade Local de Sa\u00fade da Regi\u00e3o de Leiria \u2013 Hospital de Santo Andr\u00e9, Leiria, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6602-1785","authenticated-orcid":false,"given":"Adriana","family":"Santos Silva","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Unidade Local de Sa\u00fade da Regi\u00e3o de Leiria \u2013 Hospital de Santo Andr\u00e9, Leiria, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2026,2,16]]},"reference":[{"key":"10.12890\/2026_006231_ref1","doi-asserted-by":"crossref","unstructured":"Feist E, Mitrovic S, Fautrel B. Mechanisms, biomarkers and targets for adult-onset Still\u2019s disease. Nat Rev Rheumatol 2018;14:603\u2013618. doi: 10.1038\/s41584-018-0081-x","DOI":"10.1038\/s41584-018-0081-x"},{"key":"10.12890\/2026_006231_ref2","doi-asserted-by":"crossref","unstructured":"Fautrel B, Mitrovic S, De Matteis A, Bindoli S, Anton J, Belot A, et al. EULAR\/PReS recommendations for the diagnosis and management of systemic juvenile idiopathic arthritis and adult-onset Still\u2019s disease. Ann Rheum Dis 2024;83:1614\u20131627. doi: 10.1136\/ard-2024-225851","DOI":"10.1136\/ard-2024-225851"},{"key":"10.12890\/2026_006231_ref3","unstructured":"Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H, et al. Preliminary criteria for classification of adult Still\u2019s disease. J Rheumatol 1992;19:424-430."},{"key":"10.12890\/2026_006231_ref4","doi-asserted-by":"crossref","unstructured":"Gerfaud-Valentin M, Jamilloux Y, Iwaz J, S\u00e8ve P. Adult-onset Still\u2019s disease. Autoimmun Rev 2014;13:708\u2013722. doi: 10.1016\/j.autrev.2014.01.058","DOI":"10.1016\/j.autrev.2014.01.058"}],"container-title":["European Journal of Case Reports in Internal Medicine"],"original-title":[],"link":[{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/6231\/5184","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/6231\/5185","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/download\/6231\/5185","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,3,5]],"date-time":"2026-03-05T15:07:24Z","timestamp":1772723244000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.ejcrim.com\/index.php\/EJCRIM\/article\/view\/6231"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,16]]},"references-count":4,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2026]]}},"URL":"https:\/\/doi.org\/10.12890\/2026_006231","relation":{"has-manifestation":[{"id-type":"other","id":"1-164-6231-g5184","asserted-by":"subject"},{"id-type":"other","id":"1-164-6231-g5185","asserted-by":"subject"}],"is-part-of":[{"id-type":"other","id":"1-164","asserted-by":"subject"}]},"ISSN":["2284-2594"],"issn-type":[{"value":"2284-2594","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,16]]}}}