Clinical Presentation, Management, and Long‐Term Outcome of Pars Planitis, Panuveitis, and Vogt‐Koyanagi‐Harada Disease in Children and Adolescents

Abstract

Objective. 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). The present study was undertaken to comprehensively assess the long-term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for the treatment of pediatric and adolescent patients with PP, PU, and VKH. Methods. We retrospectively reviewed a cohort of 75 children and adolescents with idiopathic PP (n = 50), PU (n = 11), and VKH (n = 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. Patients were followed for an average of 52 months. The 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%, and vision loss (<20/40) in 87% of patients, while legal blindness (≤20/200 in the better-seeing eye) was diagnosed in 18 of 75 (24%) patients (PP 22%, PU 36%, and 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 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 without medications. The worst outcome was observed in children with PU. Application of regression analysis demonstrated that young age at onset, delayed referral to a pediatric rheumatologist, and chronic disease were strong predictors for the risk of long-term blindness. Conclusion. PP, PU, and VKH involve a high risk of permanent vision loss and should be managed by a skilled rheumatologist as early and as aggressively as possible.

Comprehensive Assessment of Quality of Life, Functioning, and Mental Health in Children With Juvenile Idiopathic Arthritis and Noninfectious Uveitis

“…Further, 37% experienced one or more ocular complications, and over 40% had visual impairment during their disease course. In general, children with non‐anterior uveitis tend to have worse visual outcomes (33,34). In our cohort there were higher rates of visual impairment, ocular complications, and topical glucocorticoid use in patients with other uveitis compared to JIA‐U.…”

Adalimumab in Vogt-Koyanagi-Harada Disease Refractory to Conventional Therapy

“…Unfortunately, many VKH patients do not receive proper treatment, instead receiving delayed or inadequate therapy (suboptimal medication, premature tapering of medication or absence of immunosuppressants), and the disease inevitably progresses to the chronic recurrent stage, in which granulomatous ocular inflammation recurs and most ocular complications emerge, including complicated cataract, secondary glaucoma, choroidal neovascularization, subretinal fibrosis and others (17,(22)(23)(24). It has been indicated that ∼21% of VKH patients become legally blind (25).…”

Vogt-Koyanagi-Harada Disease and Vogt-Koyanagi-Harada Resembling Syndrome

“…Smith et al reported the presence of neurological disease to the extent of 7.9% in a large series of patients with uveitis in whom 1.1% cases were of VKH disease and 1.0% of multiple sclerosis [10]. In a study by Reiff et al to assess the long term safety and efficacy of disease-modifying antirheumatic drugs (DMARDs) and biologics for treatment of paediatric and adolescent patients with pars planitis (PP), panuveitis (PU) and VKHD, they found 98% of patients required additional DMARDs and 73% required therapy with biologics.At the end of study 35% patients across all groups had significant visual loss or were blind.They concluded that as all three groups involve a high risk of permanent visual loss they should be managed by skilled rheumatologist early and aggressively [11]. In another study by Albaroudi et al on paediatic VKHD, they concluded that life expectancy justifies early initiation of immunesuppressive or even biological therapy to achieve better steroid sparing and most importantly preserve visual function [12].…”