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Juvenile idiopathic arthritis: Epidemiology and immunopathogenesis

Alexei A Grom, MD
Section Editor
Marisa Klein-Gitelman, MD, MPH
Deputy Editor
Elizabeth TePas, MD, MS


Juvenile idiopathic arthritis (JIA) is a chronic idiopathic inflammatory disorder primarily involving joints. The epidemiology and immunopathogenesis of JIA are reviewed here. Diagnostic criteria and nomenclature of JIA are presented separately. (See "Classification of juvenile arthritis".)

Terminology — The older terms, juvenile rheumatoid arthritis (JRA, used commonly in the United States) and juvenile chronic arthritis (JCA, preferred in Europe), have been replaced by the term JIA. JIA incorporates all of what was called JRA in the past and also includes all other forms of "idiopathic" arthritis in childhood. The term JRA will still be used to describe the general patient population in older studies since JRA is a less inclusive term than JIA (the term JIA will be used for these older studies if a specific subgroup is specified [eg, systemic JIA]). However, most clinicians assume that the results of these earlier studies of JRA are generally applicable to all the categories included in JIA, although the precise numbers will be somewhat different. The JIA nomenclature should be regarded as a "work in progress" that will be refined as the genetic and pathophysiologic understanding of arthritis improves. All of the discussion of epidemiology and pathogenesis that follows is complicated by the fact that JIA is not a single disease, but several different diseases. While there are elements common to all of these diseases, some forms of JIA, such as the human leukocyte antigen (HLA)-B27-associated diseases and systemic JIA, are clearly distinct entities with differing etiologies and pathogenesis.


Overall incidence and prevalence — The lack of standard diagnostic criteria has complicated epidemiologic studies. When the revised American College of Rheumatology (ACR, formerly the ARA) classification criteria were applied to the Rochester Epidemiology Program Project database, an incidence of 13.9 cases of juvenile rheumatoid arthritis (JRA) per 100,000 per year was reported [1]. A follow-up study, utilizing the same database, noted a decrease in incidence over a subsequent decade [2].

The results of other population studies are presented in the table (table 1) [3-7]. In the Rochester study, the prevalence rate for JRA was 94 patients per 100,000 children on January 1, 1980 and 86 on January 1, 1990 [2]. An extrapolation to the entire United States population under 16 years of age in the year 2000 suggests that there must be 70,000 to 100,000 cases of JRA (active and inactive). However, the population in the Rochester study was predominantly Caucasian. The actual prevalence in the total United States population is probably much lower (15,000 to 36,000 in some estimates [8]) because JRA appears to be less common in African-American and Asian populations [9].

Ethnic differences in prevalence have also been noted in other populations. As examples, the prevalence of JRA is less in blacks than in those of Indian ancestry among South African children [10] and is higher in the native population than among Caucasians among Canadian children [11].


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