EPOS
European Paediatric Ophthalmological Society
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Eye involvement in systemic inflammatory diseases in children
Wouters Carine H.11Pediatric Rheumatology, University Hospital Gasthuisberg, Leuven, Belgium
Uveitis can be part of a variety of systemic inflammatory illness in children, most frequently juvenile idiopathic arthritis (JIA) but also pediatric sarcoidosis, Behçet’s disease, Crohn’s disease, and tubulointerstitial nephritis. In JIA, the estimated prevalence of uveitis is 17-22% of all patients; the risk of uveitis is highest in very young girls with the oligoarticular subtype of JIA and presence of antinuclear antibodies (ANA) with frequencies up to 25%. Complications of uveitis in children with JIA are numerous, including cataract and band keratopathy in up to two thirds of patients, glaucoma and impaired vision in up to one third of children. Guidelines for regular ophthalmologic evaluation comprising a high screening frequency and a long screening period have been developed. Scleritis and retinopathy can be observed in children with systemic lupus erythematosus and systemic vasculitides. Conjunctivitis is seen in acute inflammatory illnesses such as Reiter’s syndrome and Kawasaki disease. Optic disc changes associated with anterior uveitis are distinguishing features of CINCA, a hereditary autoinflammatory syndrome. Last but not least to mention are the ocular complications of systemic therapies such as corticosteroids and hydroxychlorquine. Patients with anterior uveitis may be treated with topical therapy alone but patients with posterior uveitis and those with sight threatening complications of anterior uveitis usually require systemic treatment especially if the disease is bilateral. The mainstay of treatment is corticosteroids and additional immunosuppressive agents such as cyclosporin and mycophenolate are used when necessary. There remains a significant cohort of patients in whom this therapy is either not tolerated or is ineffective. The use of the anti-tumour necrosis factor (TNF) antibodies has been very successful in controlling immune-inflammatory diseases including JIA. TNF is known to play a key role in ocular inflammation as shown by animal studies and its detection in the ocular fluids of inflamed eyes in man. The data supporting anti-TNF drugs as a potential treatment option in refractory idiopathic or JIA-associated uveitis in children are presented.