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Open Access Highly Accessed Research

Application of the Yamaguchi criteria for classification of “suspected” systemic juvenile idiopathic arthritis (sJIA)

Sharath Kumar13*, Divya Shree Kunhiraman2 and Lalitha Rajam1

Author Affiliations

1 Department of Pediatrics, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Cochin, India

2 Department of Psychiatry, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham University, Cochin, India

3 Department of Rheumatology, ISIC hospital Sector-C, Vasant Kunj, New Delhi 110070, India

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Pediatric Rheumatology 2012, 10:40  doi:10.1186/1546-0096-10-40

Published: 23 November 2012

Abstract

Background

Many children with sJIA may have a delayed onset of arthritis and so fail to fulfil the ILAR criteria for sJIA. This study was undertaken to determine whether the Yamaguchi criteria (for adult onset Still’s disease) is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in “pre-arthritic”, pure systemic, phase of the illness. A secondary objective was to determine the time delay between disease onset and onset of arthritis in our sJIA cohort.

Methods

Retrospective chart review all patients with a diagnosis of systemic juvenile arthritis in our department from Jan 1, 2004 to Jan 1, 2010.

Results

Twenty boys and eleven girls formed the study cohort. Thirteen patients were diagnosed with “suspected” sJIA due to typical systemic features but an absence of arthritis. Overall, the Yamaguchi criteria was fulfilled in a higher number of patients in the study (n=23) as compared to the ILAR criteria (n=18). Among the 13 “suspected” sJIA patients, 12 fulfilled the Yamaguchi criteria. Overall, either ILAR criteria or Yamaguchi criteria was fulfilled in 30 patients (96.8% of patients). The degree of association between the two criteria was poor (Phi coefficient = -0.352, p=0.05). Eleven out of eighteen patients with arthritis gave a history of delay in onset of arthritis (range=15 days to more than a year; median=30 days). Thus a total of 24 patients (75%) had a delay in onset of arthritis at onset of disease.

Conclusion

Patients with sJIA can have a significant period during their course (particularly at onset) when they do not have arthritis. The Yamaguchi criteria may be useful in this subset of patients in the “pre-arthritic” phase of the disease. Future criteria should incorporate the strengths of both, the Yamaguchi and the ILAR criteria.

Keywords:
Diagnosis; Arthritis; Juvenile idiopathic; Criteria; Adult-Onset Still’s disease; sJIA; Systemic JIA