Adult-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.
In 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.
Concordance 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).
In 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.
Hedrich CM, Günther C, Aringer M. Still's disease in children and adults. Z Rheumatol. 2017;76:595–608.
Yamaguchi M, Ohta A, Tsunematsu T, et al. Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992;19:424–30. PubMed
Pouchot J, Sampalis JS, Beaudet F, et al. Adult Still's disease: manifestations, disease course, and outcome in 62 patients. Medicine (Baltimore). 1991;70:118–36. CrossRef
Lebrun D, Mestrallet S, Dehoux M, et al. Validation of the Fautrel classification criteria for adult-onset Still's disease. Semin Arthritis Rheum. 2018;47:578–85.
Masson C, Le Loet X, Liote F, et al. Comparative study of 6 types of criteria in adult Still's disease. J Rheumatol. 1996;23:495–7. PubMed
Petty RE, Southwood TR, Manners P, et al. International league of associations for rheumatology classification of juvenile idiopathic arthritis: second revision, Edmonton, 2001. J Rheumatol. 2004;31:390–2. PubMed
Fink CW. Proposal for the development of classification criteria for idiopathic arthritides of childhood. J Rheumatol. 1995;22:1566–9. PubMed
Kumar S, Kunhiraman DS, Rajam L. Application of the Yamaguchi criteria for classification of "suspected" systemic juvenile idiopathic arthritis (sJIA). Pediatr Rheumatol Online J. 2012;10:40.
Luthi F, Zufferey P, Hofer MF, et al. "adolescent-onset Still's disease": characteristics and outcome in comparison with adult-onset Still's disease. Clin Exp Rheumatol. 2002;20:427–30. PubMed
Seo JY, Suh CH, Jung JY, et al. The neutrophil-to-lymphocyte ratio could be a good diagnostic marker and predictor of relapse in patients with adult-onset Still's disease: a STROBE-compliant retrospective observational analysis. Medicine (Baltimore). 2017;96:e7546. CrossRef
Chen DY, Lan JL, Lin FJ, et al. Proinflammatory cytokine profiles in sera and pathological tissues of patients with active untreated adult onset Still's disease. J Rheumatol. 2004;31:2189–98. PubMed
Sandborg C, Holmes TH, Lee T, et al. Candidate early predictors for progression to joint damage in systemic juvenile idiopathic arthritis. J Rheumatol. 2006;33:2322–9. PubMed
- Application of the international league against rheumatism classification criteria for systemic juvenile idiopathic arthritis as a prognostic factor in patients with adults-onset Still’s disease
Ji Won Yang
- BioMed Central
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