Clinical Presentation, Management and Long Term Outcome of Pars Planitis (PP), Panuveitis (PU) and Vogt-Koyanagi-Harada disease (VKH) in Children and Adolescents.

Chronic uveitis is a common manifestation of pediatric rheumatologic conditions and may result in irreversible blindness and long-term disability. While chronic anterior uveitis is the most commonly encountered ocular manifestation of rheumatic disease, little is known about the clinical presentation, management and long term outcome of more complex eye conditions such as pars planitis (PP), panuveitis (PU) and Vogt-Koyanagi-Harada disease (VKH).We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (50), PU (12) and VKH (14) followed by the Pediatric Rheumatology Core at Children's Hospital Los Angeles and evaluated referral patterns, clinical presentation, treatment response and long term clinical outcome.Patients were followed for an average of 52 months. Their mean age at disease onset was 10 years. Bilateral eye involvement was seen in 87% of the patients. At first presentation to an ophthalmologist, glaucoma was noted in 21%, vision loss (<20/40) in 87% and legal blindness (<20/200 or less in the better-seeing eye) was diagnosed in 18/75 (24%) of the patients (PP 22%, PU 36%, VKH 21%). The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Topical steroids were used in all patients, but 98% of the patients required additional DMARDs and 73% required therapy with biologics. After a mean of 52 months, 35% of patients across all disease groups had significant vision loss or were blind, and only 28% were in clinical remission off medications. The worst outcome was observed in children with panuveitis. Using regression analysis, young age at onset, delayed referral to a pediatric rheumatologist and chronic disease were strong predictors for the risk of long-term blindness.PP, PU and VKH have a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressive as possible. This article is protected by copyright. All rights reserved.