Elsevier

The Lancet

Volume 369, Issue 9563, 3–9 March 2007, Pages 767-778
The Lancet

Seminar
Juvenile idiopathic arthritis

https://doi.org/10.1016/S0140-6736(07)60363-8Get rights and content

Summary

Juvenile idiopathic arthritis is a broad term that describes a clinically heterogeneous group of arthritides of unknown cause, which begin before 16 years of age. This term encompasses several disease categories, each of which has distinct methods of presentation, clinical signs, and symptoms, and, in some cases, genetic background. The cause of disease is still poorly understood but seems to be related to both genetic and environmental factors, which result in the heterogeneity of the illness. Although none of the available drugs has a curative potential, prognosis has greatly improved as a result of substantial progresses in disease management. The most important new development has been the introduction of drugs such as anticytokine agents, which constitute a valuable treatment option for patients who are resistant to conventional antirheumatic agents. Further insights into the disease pathogenesis and treatment will be provided by the continuous advances in understanding of the mechanisms connected to the immune response and inflammatory process, and by the development of new drugs that are able to inhibit selectively single molecules or pathways.

Section snippets

Epidemiology

Juvenile idiopathic arthritis is the most common chronic rheumatic disease in children and an important cause of short-term and long-term disability. Studies in developed countries have reported a prevalence that varies between 16 and 150 per 100 000. One view is that the prevalence of this disease is underestimated. A community-based survey in Australia reported a prevalence of 400 per 100 000 on the basis of clinical examination of school children by a paediatric rheumatologist.3 Although in

Clinical features

Juvenile idiopathic arthritis classification identifies subtypes, many of which seem to represent different diseases characterised by distinct methods of presentation, clinical features, and, in some cases, genetic background.

Prognosis and outcome

Studies assessing outcome of juvenile idiopathic arthritis have provided inconsistent or conflicting results. Studies in the past 10 years have shown that only 40–60% of patients had inactive disease or clinical remission at follow-up.33, 34 A recent analysis of 437 patients followed up for at least 4 years showed that only a few (6%) episodes of clinical remission off-treatment were sustained for at least 5 years.35 Despite the long-term persistence of disease activity in most patients, a

Cause and pathogenesis

The cause and pathogenesis of juvenile idiopathic arthritis are still poorly understood but seem to include both genetic and environmental components. Moreover, the heterogeneity of this disease implies that different factors probably contribute to the pathogenesis and cause.

The notion that an infection triggers chronic arthritis in genetically susceptible individuals is attractive, but still unproven. The results of the first genome-wide scan of children with the disease lends support to the

Management

Management of juvenile idiopathic arthritis is based on a combination of pharmacological interventions, physical and occupational therapy, and psychosocial support.96, 97, 98 Although we still do not possess drugs that are able to cure the disease, prognosis has greatly improved, with respect to even a decade ago, because of substantial progresses in disease management. The aim of treatment is to reach complete control of the disease, to preserve the physical and psychological integrity of the

Future perspectives

The past decade has witnessed two major advances in assessment of the safety and effectiveness of new drugs for the treatment of paediatric rheumatic diseases.137 The first has been the implementation by the Food and Drug Administration of the so-called paediatric rule, which helps with controlled trials in childhood diseases; a similar measure has been approved by the European Parliament.138 The second has been the establishment of two large paediatric rheumatology networks, such as the

Search strategy and selection criteria

We searched the Cochrane Library, Medline, and Embase. We used the search terms “juvenile idiopathic arthritis”, “juvenile rheumatoid arthritis”, “juvenile chronic arthritis”, or “juvenile arthritis”. We largely selected publications in the past 5 years, but did not exclude commonly referenced and highly regarded older publications. We also searched the reference lists of articles identified by this search strategy and selected those we judged relevant. We paid particular attention to articles

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