Background Non-infectious uveitis is the most common cause of blindness in children. Most studies have evaluated diagnosis criteria and treatment of JIA-associated uveitis.
Objectives We analyzed treatment and outcome of childhood uveitis related to other causes than JIA.
Methods A retrospective chart review of pediatric uveitis in patients <16y-old seen between 2001 and 2015 at a university hospital in Paris. We excluded patients with JIA-associated uveitis. Clinical characteristics, treatment and ocular complications were collected. We used the SUN  (Standardization of Uveitis Nomenclature) for the classification of uveitis, disease activity (inactive uveitis: rare cells or less) and treatment endpoints, improvement: defined as a 2-step decrease in the inflammation level or a decrease to grade 0, worsening: defined as a 2-step increase in the inflammation level or an increase from grade 3+ to 4+, or remission (inactivity without treatment for ≥3 months).
Results Thirty patients (SR: 0,5; 51 eyes) were enrolled. Mean age of diagnosis was 9.6±3.5 years (3 to 15 y). Mean follow-up was 4 ±3 years. Main diagnoses were: idiopathic (60%), Behçet's disease (BD) (13%), sarcoidosis (10%), Vogt-Koyanagi– Harada syndrome (3%), herpes simplex virus (3%) and tubulointerstitial nephritis and uveitis (TINU) syndrome (3%).
Among idiopathic uveitis (n=27 eyes), 44% were anterior uveitis. Twenty eyes (74%) received systemic corticosteroid as first-line therapy, inducing inactivity for 2 eyes (10%) and remission for 1 eye (5%) at 5 years. DMARDs were needed for 18 eyes (66.6%). Methotrexate was used as first-line therapy in 14 eyes (77%) with 7.1% (n=1 eye) remission at 4 months.
Infliximab was used as second-line treatment in 9 eyes (60%) with 78% of improvement and 78% of inactivity but no remissions. Ocular complications occurred in 67% of patients: posterior synechia (37%), macular edema (37%), severe vision loss (26%), cataract (22%), vasculitis (19%), and band keratopathy (16%). In BD uveitis (n=6 eyes), 83% had panuveitis. All BD patients received systemic corticosteroid therapy and azathioprine among those 50% responded with inactive uveitis. Infliximab was used as second-line therapy in 3 eyes with 100% of improvement. All BD patients developed complications: vasculitis (83%), posterior synechiae (83%), vitreous hemorrhage (50%), severe vision loss (33%) and macular edema (17%). Sarcoidosis affected 4 eyes, 75% were panuveitis and all patients received systemic corticosteroids and became steroid dependent. All of these patients received methotrexate, and 50% required infliximab as a second line with remission in 1 eye and inactivity in 3 eyes. Posterior synechiae developed in 3 eyes.
Conclusions Idiopathic uveitis is the most common cause of non-JIA uveitis in children. BD and sarcoïdosis are thought to be the most severe ones, but idiopathic uveitis seems very difficult to treat as two third of patients have severe complications at the end of the treatment. Most patients were steroid dependent and required a DMARD in association with infliximab. Improvement and disease inactivity were frequently obtained but remission was rare.
Jabs et al., Standardization of Uveitis Nomenclature for Reporting Clinical Data. Results of the First International Workshop.
Disclosure of Interest E. Sardar: None declared, P. Dusser: None declared, M. Labetoulle: None declared, E. Barreau: None declared, B. Bodaghi: None declared, I. Kone-Paut Grant/research support from: SOBI, Roche, Consultant for: Novartis, SOBI, Pfizer, abbvie, Roche/CHUGAI