Background/Purpose:

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).

Methods:

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.

Results:

The mean age at disease onset was 10 years and patients were followed for an average of 52 months. The average referral time to a pediatric rheumatologist was 13 months (range 1-96 months). Bilateral eye involvement was seen in 87% of the patients, glaucoma was present in 21% and vision loss (<20/40) was present in 87% of the patients at first presentation. Legal blindness (<20/200 or less in the better-seeing eye) was diagnosed in 18% of the PP, 36% of the PU and 21.5% of the VKH patients at first presentation. 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, while only 28% were in clinical remission off drug. The worst outcome was observed in children with panuveitis. Earlier referral to a pediatric rheumatologist and earlier initiation of DMARD/biologic therapy clearly correlated with better outcome.

Conclusion: PP, PU and VKH have a high risk of permanent vision loss and should be managed as early and as aggressive as possible by a skilled rheumatologist.

ACR Meeting Abstracts - https://acrabstracts.org/abstract/clinical-presentation-management-and-long-term-outcome-of-pars-planitis-pp-panuveitis-pu-and-vogt-koyanagi-harada-disease-vkh-in-children-and-adolescents/