Narváez García, Francisco JavierPalacios Olid, JudithGarcía De Yebenes, María JesúsHolgado, SusanaOlivé, AlejandroCasafont Solé, IvetteCastañeda, SantosValero Martínez, CristinaMartín López, MaríaCarreira, Patricia E.Mora Limiñana, MaribelNuño Nuño, LauraRobles Marhuenda, AngelBernabeu, PilarCampos, JoséGraña, JenaroOrtiz Santamaria, VeraCamacho Lovillo, MarisolVargas, CarmenSanchez Manubens, JudithAnton, Jordi2025-03-032025-03-032025-01-062077-0383https://hdl.handle.net/2445/219392Objective: To investigate the prevalence and clinical spectrum of atypical or non-classical complications in adult-onset Still's 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's 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 1.03-7.91, p = 0.044) and the systemic score system (SSC) index (OR 1.86, 95% CI 1.29-2.69, 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 0.11-0.95, 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.12 p.application/pdfengcc-by (c) Narváez García, Francisco Javier et al., 2025http://creativecommons.org/licenses/by/3.0/es/ArtritisPronòstic mèdicArthritisPrognosisinfo:eu-repo/semantics/article2025-02-07info:eu-repo/semantics/openAccess39797367