Abstract

Pediatric patients with early onset (before the age of 6 years), antinuclear antibody positive, oligoarticular or polyarticular juvenile idiopathic arthritis (JIA), and some children with no arthritis may develop chronic, anterior uveitis. Recent recommendations insist on the need to perform slit lamp examination every 3 months for at least 5 years in early onset JIA patients in order to diagnose uveitis before complications develop. Local steroid therapy is usually the first-line treatment. However, in patients requiring steroid eye drops for several months, systemic immunomodulatory therapy is indicated. Methotrexate (MTX) is then prescribed in most cases; however, some patients also need anti-tumor necrosis factor alpha monoclonal antibody therapy and, in some cases, other biologics to control uveitis and avoid long-term ocular damage. Expert ophthalmologists and pediatricians must be involved in taking care of such patients. Immunomodulatory treatment must not be too easily interrupted and may even be intensified in some cases, particularly if there is a need for optimal disease control before ophthalmologic surgery. In good responders to MTX and/or biologics, treatment must be maintained at least 1 year, possibly even 2 years after achieving remission before tapering treatment intensity.

Highlights

  • Juvenile idiopathic arthritis (JIA) is a disease characterized by arthritis starting before the age of 16 years, lasting at least 6 weeks, and with no cause identified [1]

  • Two-thirds of JIA patients present early onset JIA, starting before the age of 6 years, with either a polyarticular or more often an oligoarticular joint involvement pattern and, in most cases, presence of non-specific antinuclear antibody (ANA) [2]. These ANA-positive, early onset JIA patients carry a risk of 20–30% chance of chronic, anterior uveitis, which almost always starts within the first 5 years of the disease

  • In JIA patients with chronic, anterior uveitis, methotrexate is recommended as the first-line systemic immunomodulatory treatment, at a dosage between 10 and 15 mg/m2 or between 0.3 and 0.6 mg/kg once a week, maximum 25 mg/week, orally or subcutaneously

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Summary

Introduction

Juvenile idiopathic arthritis (JIA) is a disease characterized by arthritis starting before the age of 16 years, lasting at least 6 weeks, and with no cause identified [1]. Two-thirds of JIA patients present early onset JIA, starting before the age of 6 years, with either a polyarticular or more often an oligoarticular (i.e., less than five arthritis within the first 6 months) joint involvement pattern and, in most cases, presence of non-specific antinuclear antibody (ANA) [2] These ANA-positive, early onset JIA patients carry a risk of 20–30% chance of chronic, anterior uveitis, which almost always starts within the first 5 years of the disease. While the development of new treatments has markedly improved the prognosis of arthritis in JIA patients, chronic uveitis may still, in the absence of early detection and effective treatment, lead to severe complications and visual impairment in a significant proportion of patients [3,4,5] In patients with another category of JIA, enthesitis-related JIA/juvenile spondylarthritis, which usually starts after the age of 6 years and mainly affects male patients, recurrent acute anterior uveitis may develop, as in adult-onset spondyloarthritis. While indicating some key recommendations from the European SHARE initiative [6] and the most recent American College of Rheumatology (ACR) recommendations [7], we will mainly refer to the most recent French recommendations for pediatric and adult-onset, chronic, non-infectious uveitis [8]

Classical Therapeutic Approaches in JIA-Associated Chronic Uveitis
Other Therapeutic Options
Some Peculiar Situations
Conclusions
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