Elsevier

Joint Bone Spine

Volume 81, Issue 2, March 2014, Pages 112-117
Joint Bone Spine

Review
Prevalence and incidence of juvenile idiopathic arthritis: A systematic review

https://doi.org/10.1016/j.jbspin.2013.09.003Get rights and content

Abstract

Objectives

To conduct a systematic literature review on incidence and prevalence of juvenile idiopathic arthritis and to estimate these figures in Europe for 2010.

Methods

Articles on incidence or prevalence of juvenile idiopathic arthritis were searched in Medline. Pooled incidence and prevalence were calculated overall, by gender, age, classification and arthritis categories. We used the available age and gender pooled rates to standardize the incidence and prevalence on the 2010 European population and estimate the number of cases in Europe in 2010.

Results

Forty-three articles (33 on incidence, 29 on prevalence) were included. Incidence rates varied from 1.6 to 23 and prevalence from 3.8 to 400/100,000. Pooled incidence and prevalence were higher for girls (10.0 [9.4–10.7] and 19.4 [18.3–20.6]/100,000) than boys (5.7 [5.3–6.2] and 11.0 [10.2–11.9]/100,000). Oligoarthritis was the most frequent form (pooled incidence rate 3.7 [3.5–3.9] and prevalence 16.8 [15.9–17.7]/100,000). The direct standardized incidence rate was 8.2 [7.5–9.0] and prevalence 70.2 [62.9–78.1]/100,000. In Europe in 2010, the estimated number of incident cases was 6896 [5481–8578] and 59,175 [44,256–76,983] prevalent cases.

Conclusions

Incidence and prevalence varied greatly among published reports of juvenile idiopathic arthritis, which may be explained by methodological issues, classification used, and time. Estimating the number of affected children can be useful, especially with the new treatment possibilities.

Introduction

Juvenile arthritis is a chronic disease affecting children and can lead, in some cases, to severe impairment. Its etiology is still unknown and its presentation is heterogeneous. Until now, no diagnostic tool exists for a positive and definite diagnosis, which is therefore difficult to obtain and is based on exclusion of other diagnosis. Different classifications of juvenile arthritis have been proposed and used over the last 3 decades. The first were the American College for Rheumatology (ACR) criteria developed in 1972 (revised 1977) [1], [2] and the European League Against Rheumatism (EULAR) criteria developed in 1977 [3]. These two classifications present similar criteria (maximal age of 16 at onset, sub-classification of the disease in pauciarticular, polyarticular and systemic arthritis), but they also have some major differences (symptom duration of 6 weeks for ACR and 3 months for EULAR, exclusion of spondylarthropathies [SPAs] for ACR). The coexistence of these 2 classifications has led to a complicated and double definition of the disease. In 1997, a working group developed a new classification, the International League of Associations for Rheumatology (ILAR) classification, which was revised in 2001 in Edmonton, Canada [4], [5]. The main criteria, components, and categories of the 3 classifications are presented in the Table S1 (see the supplementary material associated with this article online). We use juvenile idiopathic arthritis (JIA) as a generic term for juvenile chronic arthritis, juvenile rheumatoid arthritis and juvenile idiopathic arthritis and the term spondylarthropathies (SPAs) for spondylarthropathies and enthesis related arthritis.

The dynamic of a disease over time can be addressed by incidence rates, and the burden of a disease on the health care system at a point in time can be estimated by prevalence data. The incidence and prevalence of JIA have been estimated in different settings. The estimations varied greatly among studies and countries. Published literature reviews [6], [7], [8], may have identified factors explaining the observed discrepancies, which could be due to diagnostic difficulties and to the different classifications used [6], to the different means of case ascertainment and the source population [6], [7], to the changes in living conditions over time [6] and to the geographic origin [7]. Because data concerning incidence and prevalence of JIA have increased recently, especially with the new ILAR classification, a review of the latest data seems of interest. We aimed to conduct a systematic literature review of the incidence and prevalence of JIA. The secondary objective was to estimate the incidence and prevalence of this disease in Europe for 2010.

Section snippets

Search strategy

The Medline database was searched via PubMed for relevant studies by use of the terms “prevalence”, “incidence”, and “epidemiology”, combined with “juvenile rheumatoid arthritis”, “juvenile idiopathic arthritis”, “juvenile chronic arthritis” or “arthritis” and “children”. The search was limited to articles published in English, French, German or Spanish from 1972 to September 30, 2011. Abstracts of relevant studies were reviewed, and appropriate articles were then retrieved. Citations from

Results

The article selection process is shown in a flow chart (Fig. 1). The final analysis involved 43 articles, 33 concerning incidence data and 29 prevalence data (see Table S2 [11], [12], [13], [14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29], [30], [31], [32], [33], [34], [35], [36], [37], [38], [39], [40], [41], [42], [43], [44], [45], [46], [47], [48], [49], [50], [51], [52], [53], [54], [55]). The studies took place in Europe (24 articles), North

Discussion

As the last reviews on JIA epidemiology were published in 1996, 1998 and 2002, before the introduction of the ILAR classification, we aimed to perform a systematic literature review of the incidence and prevalence of JIA and estimate the incidence and prevalence in Europe in 2010. The incidence rate of JIA was variable among published reports varying from 1.6 to 42.5/100,000, with the highest rates found for indigenous children. Findings for prevalence were variable also, ranging from 3.8 to

Disclosure of interest

The authors declare that they have no conflicts of interest concerning this article.

Acknowledgments

We thank the authors of the original articles who kindly provided their data, E. Le Bihan for statistical assistance and Carolin Jourdan for her help with the forests plots.

Funding: This work was partly supported by an unconditional grant from the Association KOURIR, France.

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