Top

Pediatric Rheumatology

Published in:

Open Access 01-12-2018 | Research article

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

Authors: Ji Won Yang, Eunyoung Lee, Ji-Yeon Seo, Ju-Yang Jung, Chang-Hee Suh, Hyoun-Ah Kim

Published in: Pediatric Rheumatology | Issue 1/2018

Abstract

Background

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.

Methods

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.

Results

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).

Conclusions

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.

Bywaters EG. Still’s disease in the adult. Ann Rheum Dis. 1971;30:121–33.CrossRefPubMedPubMedCentral

Hedrich CM, Günther C, Aringer M. Still's disease in children and adults. Z Rheumatol. 2017;76:595–608.

de Graaff LC, ten Broek MR, Schweitzer DH. Is Still's disease still one disease? A case of adult-onset Still's disease showing accumulation in the carotids and the large vessels of the legs on positron emission tomography: CT images. Rheumatol Int. 2012;32:2487–90.CrossRefPubMed

Jamilloux Y, Gerfaud-Valentin M, Martinon F, et al. Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart. Immunol Res. 2015;61:53–62.CrossRefPubMed

Inoue N, Shimizu M, Tsunoda S, et al. Cytokine profile in adult-onset Still's disease: comparison with systemic juvenile idiopathic arthritis. Clin Immunol. 2016;169:8–13.CrossRefPubMed

Nigrovic PA, Raychaudhuri S, Thompson SD. Review: genetics and the classification of arthritis in adults and children. Arthritis Rheumatol. 2018;70:7–17.CrossRefPubMed

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

Nirmala N, Brachat A, Feist E, et al. Gene-expression analysis of adult-onset Still's disease and systemic juvenile idiopathic arthritis is consistent with a continuum of a single disease entity. Pediatr Rheumatol Online J. 2015;13:50.CrossRefPubMedPubMedCentral

10.

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.

11.

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

12.

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

13.

Fink CW. Proposal for the development of classification criteria for idiopathic arthritides of childhood. J Rheumatol. 1995;22:1566–9.PubMed

14.

Ravelli A, Varnier GC, Oliveira S, et al. Antinuclear antibody-positive patients should be grouped as a separate category in the classification of juvenile idiopathic arthritis. Arthritis Rheum. 2011;63:267–75.CrossRefPubMed

15.

Martini A. It is time to rethink juvenile idiopathic arthritis classification and nomenclature. Ann Rheum Dis. 2012;71:1437–9.CrossRefPubMed

16.

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.

17.

Landis JR, Koch GG. An application of hierarchical kappa-type statistics in the assessment of majority agreement among multiple observers. Biometrics. 1977;33:363–74.CrossRefPubMed

18.

Lin SJ, Chao HC, Yan DC. Different articular outcomes of Still's disease in Chinese children and adults. Clin Rheumatol. 2000;19:127–30.CrossRefPubMed

19.

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

20.

Uppal SS, Pande IR, Kumar A, et al. Adult onset Still's disease in northern India: comparison with juvenile onset Still's disease. Br J Rheumatol. 1995;34:429–34.CrossRefPubMed

21.

Han JH, Suh CH, Jung JY, et al. Association of CXCL10 and CXCL13 levels with disease activity and cutaneous manifestation in active adult-onset Still's disease. Arthritis Res Ther. 2015;17:260.CrossRefPubMedPubMedCentral

22.

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

23.

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

24.

Ruscitti P, Cipriani P, Masedu F, et al. Adult-onset Still's disease: evaluation of prognostic tools and validation of the systemic score by analysis of 100 cases from three centers. BMC Med. 2016;14:194.CrossRefPubMedPubMedCentral

25.

Russo RA, Katsicas MM. Patients with very early-onset systemic juvenile idiopathic arthritis exhibit more inflammatory features and a worse outcome. J Rheumatol. 2013;40:329–34.CrossRefPubMed

26.

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

Title: 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
Authors: Ji Won Yang
Eunyoung Lee
Ji-Yeon Seo
Ju-Yang Jung
Chang-Hee Suh
Hyoun-Ah Kim
Publication date: 01-12-2018
Publisher: BioMed Central
Published in: Pediatric Rheumatology / Issue 1/2018
Electronic ISSN: 1546-0096
DOI: https://doi.org/10.1186/s12969-018-0225-1

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Background

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

Please log in to get access to this content

Other articles of this Issue 1/2018

Progressive pseudorheumatoid dysplasia with new-found gene mutation of Wntl inducible signaling pathway protein 3

Successful implementation of a clinical transition pathway for adolescents with juvenile-onset rheumatic and musculoskeletal diseases

Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus

IL-6 blockade in systemic juvenile idiopathic arthritis – achievement of inactive disease and remission (data from the German AID-registry)

Reliability of ultrasonography to detect inflammatory lesions and structural damage in juvenile idiopathic arthritis

Coronary artery status of patients with transient fever 24–36 h after first IVIG infusion did not differ from that seen in responsive patients