Abstract
Abstract Systemic juvenile idiopathic arthritis (sJIA) constitutes a small part of juvenile idiopathic arthritis (JIA), yet has a disproportionally higher rate of mortality. In comparison to the other (JIA), systemic juvenile idiopathic arthritis has many distinct clinical features that arthritis may be a non-prominent and/or a delayed manifestation. The Pediatric Rheumatology International Trial Organization (PRINTO), currently is developing a new criterion for JIA classification which will allow for better identification of sJIA cases with delayed onset of arthritis and fail to fulfil the currently used International League Against Rheumatism (ILAR) and Yamaguchi criteria. It is useful in classification of children with systemic juvenile idiopathic arthritis (sJIA) particularly in ‘pre-arthritic’, pure systemic phase of the illness. Objectives The purpose of this study is to compare the performances of the Yamaguchi diagnostic criteria vs PRINTO criteria vs current ILAR criteria for systemic juvenile idiopathic arthritis. We compared the proportions of patients classified and how closely the 3 instruments are related. Methods The study was performed at the Department of Paediatric Rheumatology in Tripoli children hospital. It is a retrospective study, covering the date range 2006–2020. We used clinicobiologic data recorded within 7 months of diagnosis of Patients diagnosed with sJIA. We assigned a diagnosis of sJIA according to the 3 sJIA classification systems (the Yamaguchi diagnostic criteria, PRINTO criteria and according to ILAR classification). Results Three different classification criteria were analysed in 38 assigned sJIA children (13% of total JIA cases). Females were 28 (52.6%), and mean age of JIA onset was 7 ± 3.9 yr. The mean of the time delay between disease onset and diagnosis was 7 ± 9 months. Overall, the ILAR and PRINTO criteria were fulfilled in a higher number of patients in the study (n = 35) (92% of patients), As compared with the Yamaguchi criteria (n = 29).(76% of patients). Among the 7.9% patients who were unclassifiable using the ILAR criteria were diagnosed as sJIA by both the PRINTO and the Yamaguchi diagnostic criteria. 7.89% neither fulfilled the Yamaguchi or the PRINTO Criteria. The table below compares the performance of the three different criteria. Conclusions In this cohort the ILAR criteria appear to be superior in the early recognition and classification of sJIA. Yamaguchi diagnostic criteria can only be used to classify a minority of sJIA patients, leaving a large proportion of sJIA patients unclassified. Compared with the Yamaguchi criteria, ILAR JIA categories aligned better with PRINTO criteria. All the criteria should incorporate strengths of each other. Ethics Our study was approved by local ethical committee of pediatric department at Tripoli children hospital.
Published Version
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