Abstract
BackgroundAdult-onset Still’s disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. This study evaluated the concordance between the International League Against Rheumatism (ILAR) criteria for systemic JIA and the Yamaguchi criteria and then compared their possible prognostic value in patients with AOSD.MethodsIn a retrospective review of 169 adults with suspected AOSD, patients were classified according to the Yamaguchi or ILAR criteria. Then the concordance in cross-referencing the other group with the different criteria was investigated and the sensitivity and specificity of each set of criteria were determined. Disease activity markers in AOSD patients were correlated with positivity according to both systems.ResultsConcordance was good in patients with suspected AOSD (k = 0.7144, p < 0.001) and low in those with a diagnosis of AOSD (k = 0.3787, p < 0.001). The sensitivity of the ILAR criteria in AOSD patients was 0.8864 (95% confidence interval (CI): 0.8322–0.9405), and the specificity was 0.7838 (0.6511, 0.9164). Positivity according to the ILAR criteria correlated with the systemic score (r = 0.763, p < 0.0001) and C-reactive protein levels (r = 0.183, p = 0.0356) and was associated with a relapse (odds ratio: 1.589, 95% CI: 1.043–2.421), macrophage activation syndrome (MAS; odds ratio: 1.993, 95% CI: 1.218–3.263) and care in the intensive care unit (ICU; odds ratio: 2.087, 95% CI: 1.086–4.011).ConclusionsIn AOSD patients, there is fair concordance between the Yamaguchi and ILAR criteria for systemic JIA. Positive ILAR criteria may be useful for identifying AOSD patients at high risk for relapse, MAS and the need for ICU care. Further studies including larger populations from several centers are needed to confirm our results regarding the utility of the ILAR criteria in AOSD patients.
Highlights
Adult-onset Still’s disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification
Individuals presenting at the age of 16 years and older are diagnosed with adult-onset Still’s disease (AOSD); younger individuals are diagnosed with systemic JIA [2, 3]
12 patients were diagnosed with palindromic rheumatism, 11 with lupus-like disease, 4 with viral exanthema, 2 each with drug eruptions, Kikuchi’s disease, and systemic lupus erythematosus, and 1 each with breast cancer, thyroid cancer, human immunodeficiency virus infection, and hypereosinophilic syndrome
Summary
Adult-onset Still’s disease (AOSD) is an adult form of systemic juvenile idiopathic arthritis (JIA) that differs from the latter in its classification. Still’s disease is a systemic form of juvenile idiopathic arthritis (JIA), the etiology and pathogenesis of which are unknown. A cohort of 14 adults who presented with the same symptoms as JIA patients was described in 1971, and the name adult-onset Still’s disease (AOSD) was proposed [1]. A recent study compared the cytokine profiles of patients with AOSD and systemic JIA [5]. Differences between the IL6- and IL-18-based cytokine profiles may be responsible for the different clinical manifestations of JIA and AOSD and suggest the presence of two distinct subgroups within a single disease category [6]
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