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Juvenile Idiopathic Arthritis-Associated Uveitis
Key Concepts
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Early diagnosis of juvenile idiopathic arthritis improves ocular morbidity and long-term outcome.
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Despite growing understanding of immunopathogenic mechanisms, the etiology of juvenile idiopathic arthritis remains speculative.
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Frequent ocular examination of high-risk juveniles is key to early initiation of treatment.
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Despite intensive screening programs, juvenile idiopathic arthritis-associated uveitis remains a blinding disease if not recognized early and treated aggressively.
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Aggressive control of intraocular inflammation reduces complications and improves outcomes.
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Corticosteroid therapy in children is associated with significant risks of development of cataract and glaucoma.
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Better anatomic and visual outcomes may be obtained by limiting steroid use and by early institution of steroid-sparing immunomodulatory therapy.
Juvenile idiopathic arthritis (JIA) is a heterogeneous group of arthritides of unknown origin beginning before the age of 16 and lasting for 6 or more weeks. Multiple classification schemes for the pediatric arthropathies have been published. Most have been superseded by the International League of Associations of Rheumatology (ILAR) classification published in 2004. Features present in the first 6 months of illness lead to the description of seven disease categories: oligoarthritis, rheumatoid-factor (RF)-positive polyarthritis, RF-negative polyarthritis, enthesitis-related arthritis (ERA), psoriatic arthritis, systemic arthritis, and undifferentiated arthritis. The terms juvenile rheumatoid arthritis and juvenile chronic arthritis, used in North America and Europe respectively, were officially replaced with JIA. JIA is the most common chronic rheumatic disease of childhood and a leading cause of short- and long-term disability. While arthralgia and stiffness are major clinical features of JIA, extraarticular manifestations are not negligible. The most common extraarticular manifestation of JIA is uveitis, particularly anterior uveitis.
Epidemiology
The results of a review of 34 epidemiologic studies established that the prevalence of JIA varies from 0.07 to 4.01 per 1000 children and the incidence from 0.008 to 0.226 per 1000 children, corresponding respectively to a greater than 50-fold and 28-fold difference between the extremes. The heterogeneous nature of the disease, the different classification criteria as well as the difference in case ascertainment make data on incidence and prevalence vary greatly. JIA occurs worldwide but has been mostly studied in North American and European populations. In the United States and Canada, the incidence of JIA is estimated at 0.041–0.061 per 1000 children. Over observation periods of 12–17 years, studies recorded a doubling or tripling of the incidence of JIA, supporting possible environmental risk factors for the disease. JIA affects children of all races and ages, females more than males, but remarkable demographic differences are seen between JIA subtypes. Oligoarthritis is the most common subtype of JIA encountered in North America and Europe, whereas polyarthritis predominates in most parts of Asia (Japan, China, and India) and South Africa. The peak age of onset for oligoarticular JIA is between 2 and 4 years old while RF-negative polyarticular JIA has a biphasic distribution with an early peak at 2–4 years and later peak at 6–12 years. Susceptibility to JIA is inherited, and siblings of patients with JIA are 15–30-fold more likely than the general population to develop JIA.
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