22 - Uveitis in Juvenile Idiopathic Arthritis

Abstract

Uveitis is a potentially sight-threatening complication of pediatric rheumatic diseases, in particular juvenile idiopathic arthritis (JIA). The most common form of uveitis seen in association with JIA is chronic anterior uveitis, which is almost always asymptomatic in the initial stages. Therefore screening for JIA-associated uveitis in at-risk patients is essential. The aim is to detect the disease early and commence treatment before development of visually disabling complications. These include cataracts, glaucoma, band keratopathy, and cystoid macular edema, and can result from uncontrolled intraocular inflammation and its treatment, particularly prolonged high-dose topical corticosteroids. Evidence-based consensus recommendations support early introduction of systemic immunosuppressants, such as methotrexate, as steroid-sparing agents. Two randomized controlled trials provide convincing evidence for the use of adalimumab in patients who fail to respond adequately to methotrexate. Tocilizumab has also been investigated as an alternative in children inadequately treated by antitumor necrosis factor drugs. The questions of when and how to wean systemic immunosuppression once uveitis is in remission remain unanswered. Consensus recommendations suggest continuing treatment for at least 24 months of inactive disease. Observational studies, however, have reported uveitis reactivation in over 60% of patients after withdrawing systemic therapy. Future research should aim to identify biomarkers, which can predict children at high risk of developing JIA-associated uveitis or likely to relapse off treatment. Ultimately the aim is to target patients to appropriate treatment and therefore reduce visual loss and ocular morbidity.