Application of the Yamaguchi criteria for classification of -suspected- systemic juvenile idiopathic arthritis sJIAReport as inadecuate

Application of the Yamaguchi criteria for classification of -suspected- systemic juvenile idiopathic arthritis sJIA - Download this document for free, or read online. Document in PDF available to download.

Pediatric Rheumatology

, 10:40

First Online: 23 November 2012Received: 05 March 2012Accepted: 10 November 2012


BackgroundMany 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.

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

ResultsTwenty 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.

ConclusionPatients 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.

KeywordsDiagnosis Arthritis Juvenile idiopathic Criteria Adult-Onset Still’s disease sJIA Systemic JIA AbbreviationsAOSDAdult onset still’s disease

DMARDDisease modifying anti-rheumatic drugs

ILARInternational league of associations for rheumatology

FUOFever of unknown origin

sJIASystemic juvenile idiopathic arthritis.

Electronic supplementary materialThe online version of this article doi:10.1186-1546-0096-10-40 contains supplementary material, which is available to authorized users.

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Author: Sharath Kumar - Divya Shree Kunhiraman - Lalitha Rajam


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