{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,26]],"date-time":"2026-08-26T08:12:32Z","timestamp":1787731952892,"version":"build-2784847793"},"reference-count":33,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2025,1,6]],"date-time":"2025-01-06T00:00:00Z","timestamp":1736121600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Spanish Society for Paediatric Rheumatology (SERPE)"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objective: To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still\u2019s disease (AOSD) beyond macrophage activation syndrome (MAS) and to identify factors linked to their occurrence. Methods: Multicenter cross-sectional study of AODS cases included in the Spanish registry on Still\u2019s disease. Results: This study included 107 patients (67% women), of whom 64 (59.8%) developed non-classical complications. These include macrophage activation syndrome in 9.5%, atypical skin manifestations in 38.8%, cardiac involvement in 22.7% (comprising pericarditis, myocarditis, pulmonary arterial hypertension, and noninfectious endocarditis), pleuritis in 28.9%, transient pulmonary infiltrates in 4%, significant headache in 14.1%, lower abdominal pain with evidence of peritonitis in 8.4%, and secondary amyloidosis in 0.9%. In the multivariate logistic regression analysis, lymphadenopathy (OR 2.85, 95% CI 1.03\u20137.91, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29\u20132.69, p = 0.001) were independently associated with the development of non-classical clinical manifestations. In contrast, typical exanthema was associated with a reduced risk of these complications (OR 0.32, 95% CI 0.11\u20130.95, p = 0.041). Conclusions: In addition to the typical clinical manifestations and MAS, a significant proportion of patients with AOSD develop uncommon complications, some of which can be potentially life-threatening. These should be considered in the evaluation and follow-up of patients. Early recognition and prompt management are crucial to significantly reduce morbidity and mortality.<\/jats:p>","DOI":"10.3390\/jcm14010285","type":"journal-article","created":{"date-parts":[[2025,1,6]],"date-time":"2025-01-06T08:08:52Z","timestamp":1736150932000},"page":"285","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Non-Classical Complications of Adult-Onset Still\u2019s Disease: A Multicenter Spanish Study"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1614-8064","authenticated-orcid":false,"given":"Javier","family":"Narv\u00e1ez","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Judith","family":"Palacios-Olid","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mar\u00eda Jes\u00fas","family":"Garc\u00eda de Yebenes","sequence":"additional","affiliation":[{"name":"Instituto de Salud Musculoesquel\u00e9tica, 28045 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susana","family":"Holgado","sequence":"additional","affiliation":[{"name":"Hospital Universitari Germans Trias i Pujol, 08916 Badalona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alejandro","family":"Oliv\u00e9","sequence":"additional","affiliation":[{"name":"Hospital Universitari Germans Trias i Pujol, 08916 Badalona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1004-300X","authenticated-orcid":false,"given":"Ivette","family":"Casafont-Sol\u00e9","sequence":"additional","affiliation":[{"name":"Hospital Universitari Germans Trias i Pujol, 08916 Badalona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Santos","family":"Casta\u00f1eda","sequence":"additional","affiliation":[{"name":"Hospital Universitario de La Princesa, IIS-Princesa, C\u00e1tedra UAM-Roche, Universidad Aut\u00f3noma de Madrid, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2013-1545","authenticated-orcid":false,"given":"Cristina","family":"Valero-Mart\u00ednez","sequence":"additional","affiliation":[{"name":"Hospital Universitario de La Princesa, IIS-Princesa, C\u00e1tedra UAM-Roche, Universidad Aut\u00f3noma de Madrid, 28006 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4218-5731","authenticated-orcid":false,"given":"Mar\u00eda","family":"Mart\u00edn-L\u00f3pez","sequence":"additional","affiliation":[{"name":"Hospital Universitario 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8279-3806","authenticated-orcid":false,"given":"Patricia E.","family":"Carreira","sequence":"additional","affiliation":[{"name":"Hospital Universitario 12 de Octubre, 28041 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maribel","family":"Mora-Limi\u00f1ana","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Hospital Universitario de Bellvitge, Bellvitge Biomedical Research Institute (IDIBELL), 08907 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Nu\u00f1o-Nu\u00f1o","sequence":"additional","affiliation":[{"name":"Hospital Universitario La Paz, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1156-2570","authenticated-orcid":false,"given":"Angel","family":"Robles-Marhuenda","sequence":"additional","affiliation":[{"name":"Hospital Universitario La Paz, 28046 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pilar","family":"Bernabeu","sequence":"additional","affiliation":[{"name":"Hospital General Universitario de Alicante, 03010 Alicante, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1744-0910","authenticated-orcid":false,"given":"Jos\u00e9","family":"Campos","sequence":"additional","affiliation":[{"name":"Hospital Universitario Puerta de Hierro, 28222 Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9041-8117","authenticated-orcid":false,"given":"Jenaro","family":"Gra\u00f1a","sequence":"additional","affiliation":[{"name":"Complejo Hospitalario Universitario de A Coru\u00f1a, 15006 A Coru\u00f1a, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vera","family":"Ortiz-Santamaria","sequence":"additional","affiliation":[{"name":"Hospital General de Granollers, 08402 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marisol","family":"Camacho-Lovillo","sequence":"additional","affiliation":[{"name":"Hospital Universitario Virgen del Roc\u00edo, 41013 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carmen","family":"Vargas","sequence":"additional","affiliation":[{"name":"Hospital Universitario Virgen Macarena, 41009 Sevilla, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7615-4765","authenticated-orcid":false,"given":"Judith","family":"Sanchez-Manubens","sequence":"additional","affiliation":[{"name":"Hospital Universitari Parc Taul\u00ed, 08208 Sabadell, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8792-4219","authenticated-orcid":false,"given":"Jordi","family":"Anton","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology, Institut de Recerca Sant Joan de D\u00e9u, Hospital Sant Joan de D\u00e9u, Universitat de Barcelona, 08950 Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,1,6]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"116","DOI":"10.1038\/s41584-023-01065-6","article-title":"Recent advances and evolving concepts in Still\u2019s 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