Overview
Vogt-Koyanagi-Harada (VKH) disease is a multisystem inflammatory disorder characterized by bilateral uveitis, often accompanied by neurological and auditory symptoms, and can mimic other conditions like papilledema due to Arnold Chiari malformation 1.Diagnosis
Key Clinical Features: Bilateral uveitis, serous retinal detachment, optic disc edema, vitritis, and anterior chamber inflammation 13.
Neurological and Auditory Symptoms: Headaches, tinnitus, hearing impairment, and polyarthritis may be present 23.
Diagnostic Tests: Comprehensive ophthalmic examination including fundus imaging, visual field testing, and neuroimaging if neurological signs are present 1.
Grading: Clinical criteria often used for diagnosis include the presence of bilateral uveitis, optic disc edema, and systemic manifestations; specific grading systems may vary 3.Management
First-Line Treatment: High-dose intravenous corticosteroids (e.g., methylprednisolone) followed by oral corticosteroids 1.
Adjunctive Therapy: Immunosuppressive agents such as methotrexate, azathioprine, or mycophenolate mofetil for refractory cases 1.
Monitoring: Regular ophthalmic follow-up to assess response to treatment and manage relapses 3.Special Populations
Pediatrics: VKH is rare in children but can present with chronic diffuse uveitis or neurological symptoms; management follows adult protocols with close monitoring 4.
Comorbidities: Cases may overlap with conditions like Arnold Chiari malformation, necessitating multidisciplinary care 1.Key Recommendations
Perform thorough ophthalmic evaluation in cases of bilateral optic disc edema to differentiate VKH from other causes like papilledema 1 (Evidence: Moderate).
Initiate treatment with high-dose intravenous corticosteroids for acute VKH, transitioning to oral corticosteroids and adjunctive immunosuppression if necessary 1 (Evidence: Moderate).
Monitor for and manage potential neurological and auditory manifestations alongside ocular symptoms in VKH patients 23 (Evidence: Moderate).References
1 Agrawal A, Gangwe AB, Singh A, Shrivastava P, Sushmitha S, Agrawal D. Vogt Koyanagi Harada disease with bilateral disc edema diagnosed as Arnold Chiari malformation with papilledema: The blurred margin between ophthalmology and neurology. Indian journal of ophthalmology 2025. link
2 Oliveira D, Martins A, Costa L, Vaz C. Vogt-Koyanagi-Harada Syndrome and polyarthritis as a rare clinical manifestation. Acta reumatologica portuguesa 2021. link
3 Guayacán CL, Galindo-Mendez B, de-la-Torre A. Vogt-Koyanagi-Harada Syndrome in a Group of Patients in Two Ophthalmology Referral Centers in Bogotá, Colombia. Ocular immunology and inflammation 2018. link
4 Weber SW, Kazdan JJ. The Vogt--Koyanagi--Harada syndrome in children. Journal of pediatric ophthalmology 1977. link