P0903 - Vogt-Koyanagi-Harada Disease in a Pediatric Patient

Introduction
Vogt-Koyanagi-Harada (VKH) disease is a systematic autoimmune condition characterized by bilateral non-necrotizing granulomatous panuveitis and extraocular manifestations. Although rare in the pediatric patient, cases of VKH appear to be more aggressive in children compared to in adults and may cause irreversible vision loss.

Case Description
A 16-year-old Hispanic female with no significant past medical history was referred to the emergency room for papilledema and macular edema. Her initial diagnostic examination was significant for cerebrospinal fluid pleocytosis, with an opening pressure within normal limits. The patient was therefore admitted to the hospital for papilledema and meningitis of unknown etiology.

During her hospitalization, the patient continued to have worsening vision despite the initiation of antibiotic and antiviral medications. Additional imaging studies also revealed findings concerning for chorioretinitis (Figure 1). High-dose steroids were started to prevent further vision loss. The patient displayed good clinical response to the steroids, with noted improvement of her vision. However, her bloodwork at this time revealed positive immunoglobulin G titers for toxoplasmosis. Because the patient was on high-dose steroids, there were concerns as to whether treatment was needed for toxoplasma. The decision was made that treatment was not indicated due to negative immunoglobulin M titers, clinical improvement while on steroids, and the lack of other findings suggestive of an active toxoplasma infection. Antibiotics and antivirals were discontinued as all cultures remained negative. The patient was medically stable and referred for further outpatient evaluation by ophthalmology. During her outpatient ophthalmology follow-up, the patient showed signs of retinal detachment. Based on her hospital course and clinical symptoms, the patient was diagnosed with incomplete VKH disease. She continued on a course of high-dose steroids with adalimumab added on as an adjunct therapy.

Discussion
The patient in this case was diagnosed outpatient with incomplete VKH disease based on VKH committee’s current diagnostic criteria (Table 1). The lack of integumentary findings precluded her from a complete disease diagnosis. The diagnosis of VKH disease in this specific case was difficult to make due to laboratory results suggestive of an infectious process and the rarity of this condition in the pediatric population.

Conclusion
VKH disease is uncommon in pediatrics and can be aggressive and potentially vision-threatening. Current literature has noted fewer than 200 cases of VKH in children under 17 years old. The exact pathogenesis of VKH disease remains unclear, but is thought to be a T-cell mediated autoimmune reaction against melanocyte antigens. Timely induction of steroid therapy is important to ensure good visual prognosis. Currently, adalimumab remains an off-label treatment for pediatric patients with non-infectious uveitis. Further research is warranted to establish a systematic approach to the management of pediatric VKH.