The Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry

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dc.contributor.authorRuscitti P.
dc.contributor.authorMasedu F.
dc.contributor.authorVitale A.
dc.contributor.authorCaggiano V.
dc.contributor.authorDi Cola I.
dc.contributor.authorCipriani P.
dc.contributor.authorValenti M.
dc.contributor.authorMayrink Giardini H.A.
dc.contributor.authorde Brito Antonelli I.P.
dc.contributor.authorDagostin M.A.
dc.contributor.authorLopalco G.
dc.contributor.authorIannone F.
dc.contributor.authorMaria M.
dc.contributor.authorAlmaghlouth I.A.
dc.contributor.authorAsfina K.N.
dc.contributor.authorAli H.H.
dc.contributor.authorCiccia F.
dc.contributor.authorIacono D.
dc.contributor.authorPantano I.
dc.contributor.authorMauro D.
dc.contributor.authorSfikakis P.P.
dc.contributor.authorTektonidou M.
dc.contributor.authorLaskari K.
dc.contributor.authorBerardicurti O.
dc.contributor.authorDagna L.
dc.contributor.authorTomelleri A.
dc.contributor.authorTufan A.
dc.contributor.authorCan Kardas R.
dc.contributor.authorHinojosa-Azaola A.
dc.contributor.authorMartín-Nares E.
dc.contributor.authorKawakami-Campos P.A.
dc.contributor.authorRagab G.
dc.contributor.authorHegazy M.T.
dc.contributor.authorDireskeneli H.
dc.contributor.authorAlibaz-Oner F.
dc.contributor.authorFotis L.
dc.contributor.authorSfriso P.
dc.contributor.authorGovoni M.
dc.contributor.authorLa Torre F.
dc.contributor.authorCristina Maggio M.
dc.contributor.authorMontecucco C.
dc.contributor.authorDe Stefano L.
dc.contributor.authorBugatti S.
dc.contributor.authorRossi S.
dc.contributor.authorMakowska J.
dc.contributor.authorDel Giudice E.
dc.contributor.authorEmmi G.
dc.contributor.authorBartoloni E.
dc.contributor.authorHernández-Rodríguez J.
dc.contributor.authorConti G.
dc.contributor.authorNunzia Olivieri A.
dc.contributor.authorLo Gullo A.
dc.contributor.authorSimonini G.
dc.contributor.authorViapiana O.
dc.contributor.authorWiesik-Szewczyk E.
dc.contributor.authorErten S.
dc.contributor.authorCarubbi F.
dc.contributor.authorDe Paulis A.
dc.contributor.authorMaier A.
dc.contributor.authorTharwat S.
dc.contributor.authorCosti S.
dc.contributor.authorIagnocco A.
dc.contributor.authorSebastiani G.D.
dc.contributor.authorGidaro A.
dc.contributor.authorBrucato A.L.
dc.contributor.authorKaramanakos A.
dc.contributor.authorAkkoç N.
dc.contributor.authorCaso F.
dc.contributor.authorCosta L.
dc.contributor.authorPrete M.
dc.contributor.authorPerosa F.
dc.contributor.authorAtzeni F.
dc.contributor.authorGuggino G.
dc.contributor.authorFabiani C.
dc.contributor.authorFrediani B.
dc.contributor.authorGiacomelli R.
dc.contributor.authorCantarini L.
dc.date.accessioned2025-04-10T11:02:19Z
dc.date.available2025-04-10T11:02:19Z
dc.date.issued2024
dc.description.abstractObjective: We aimed to evaluate the clinical usefulness of the systemic score in the prediction of life-threatening evolution in Still disease. We also aimed to assess the clinical relevance of each component of the systemic score in predicting life-threatening evolution and to derive patient subsets accordingly. Methods: A multicenter, observational, prospective study was designed including patients included in the Gruppo Italiano Di Ricerca in Reumatologia Clinica e Sperimentale Adult-Onset Still Disease Study Group and the Autoinflammatory Disease Alliance Network Still Disease Registry. Patients were assessed to see if the variables to derive the systemic score were available. The life-threatening evolution was defined as mortality, whatever the clinical course, and/or macrophage activation syndrome, a secondary hemophagocytic lymphohistiocytosis associated with a poor prognosis. Results: A total of 597 patients with Still disease were assessed (mean ± SD age 36.6 ± 17.3 years; male 44.4%). The systemic score, assessed as a continuous variable, significantly predicted the life-threatening evolution (odds ratio [OR] 1.24; 95% confidence interval [CI] 1.07–1.42; P = 0.004). A systemic score ≥7 also significantly predicted the likelihood of a patient experiencing life-threatening evolution (OR 3.36; 95% CI 1.81–6.25; P < 0.001). Assessing the clinical relevance of each component of the systemic score, liver involvement (OR 1.68; 95% CI 1.48–2.67; P = 0.031) and lung disease (OR 2.12; 95% CI 1.14–4.49; P = 0.042) both significantly predicted life-threatening evolution. The clinical characteristics of patients with liver involvement and lung disease were derived, highlighting their relevance in multiorgan disease manifestations. Conclusion: The clinical utility of the systemic score was shown in identifying Still disease at a higher risk of life-threatening evolution in a large cohort. Furthermore, the clinical relevance of liver involvement and lung disease was highlighted. (Figure presented.). © 2024 American College of Rheumatology.
dc.identifier.DOI-ID10.1002/art.42845
dc.identifier.urihttp://hdl.handle.net/20.500.14701/43975
dc.publisherJohn Wiley and Sons Inc
dc.titleThe Systemic Score May Identify Life-Threatening Evolution in Still Disease: Data from the GIRRCS AOSD-Study Group and the AIDA Network Still Disease Registry
dc.typeArticle

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