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
SeminarJuvenile idiopathic arthritis
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
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