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

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.

Figure 1

The percentage of patients fulfilling the different component criterion of the ILAR and Yamaguchi criteria in our cohort. Actual numbers of patients are in brackets.

Figure 2

Figure depicting the delay between onset of fever and onset of arthritis in the eighteen patients who fulfilled ILAR criteria at presentation to our department. Cumulative number of patients with arthritis is plotted on the y axis against the number of days (in geometric progression) since they developed fever on the x-axis.

Figure 3

Characteristics of patients who fulfilled the ILAR criteria but failed to fulfil the Yamaguchi criteria. (Total n=7) All patients had fever and arthritis.(components of both Yamaguchi and ILAR criteria). None of the patients had a positive ANA nor RF (Component of Yamaguchi criteria). Further components of Yamaguchi criteria are in black and of the ILAR criteria are in grey.