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.accessioned2024-07-22T08:01:09Z
dc.date.available2024-07-22T08:01:09Z
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.issn23265191
dc.identifier.urihttp://akademikarsiv.cbu.edu.tr:4000/handle/123456789/11349
dc.language.isoEnglish
dc.publisherJohn Wiley and Sons Inc
dc.subjectAdult
dc.subjectDisease Progression
dc.subjectFemale
dc.subjectHumans
dc.subjectMacrophage Activation Syndrome
dc.subjectMale
dc.subjectMiddle Aged
dc.subjectPrognosis
dc.subjectProspective Studies
dc.subjectRegistries
dc.subjectSeverity of Illness Index
dc.subjectStill's Disease, Adult-Onset
dc.subjectYoung Adult
dc.subjectanakinra
dc.subjectantirheumatic agent
dc.subjectbiological product
dc.subjectcanakinumab
dc.subjectdisease modifying antirheumatic drug
dc.subjectglucocorticoid
dc.subjecttocilizumab
dc.subjecttumor necrosis factor inhibitor
dc.subjectabdominal pain
dc.subjectadult
dc.subjectArticle
dc.subjectclinical significance
dc.subjectcohort analysis
dc.subjectdisease association
dc.subjectdisease course
dc.subjectdisease registry
dc.subjectfemale
dc.subjecthuman
dc.subjectliver disease
dc.subjectlow drug dose
dc.subjectlung disease
dc.subjectlymphadenopathy
dc.subjectmacrophage activation syndrome
dc.subjectmajor clinical study
dc.subjectmale
dc.subjectmortality
dc.subjectmortality rate
dc.subjectmulticenter study (topic)
dc.subjectmultiple organ failure
dc.subjectobservational study
dc.subjectpericarditis
dc.subjectpleurisy
dc.subjectprognosis
dc.subjectscoring system
dc.subjectsecondary hemophagocytic lymphohistiocytosis
dc.subjectserositis
dc.subjectsore throat
dc.subjectsplenomegaly
dc.subjectsystemic juvenile idiopathic arthritis
dc.subjectadult onset Still disease
dc.subjectclinical trial
dc.subjectcomplication
dc.subjectdiagnosis
dc.subjectdisease exacerbation
dc.subjectmiddle aged
dc.subjectmulticenter study
dc.subjectprospective study
dc.subjectregister
dc.subjectseverity of illness index
dc.subjectyoung adult
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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