TITLE:
Vogt-Koyanagi-Harada (VKH) Disease
AUTHORS:
Jyoti Amal Upadhyay, Parth Hemantkumar Joshi, Sabia Afreen, Safiya Mohamed Ramzy
KEYWORDS:
VKH Syndrome, VKH Disease, Vogt Koyanagi Harada
JOURNAL NAME:
Open Journal of Clinical Diagnostics,
Vol.14 No.3,
September
20,
2024
ABSTRACT: Background: Vogt-Koyanagi-Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by bilateral granulomatous panuveitis, with or without extraocular manifestations. Although its exact etiology and pathogenesis remain unclear, it is hypothesized to involve T-cell dysregulation targeting melanocyte-containing tissues, including the CNS, eye, ear, and skin. VKH predominantly affects pigmented groups, such as Asians, Hispanics, Indians, Native Americans, and Mediterranean ethnicities, accounting for 7-22.4% of uveitis cases. Retrospective analyses indicate a higher incidence among female patients, with most cases occurring in the second and fifth decades of life. Aim: This case report discusses a patient with probable VKH who exhibited ocular, neurologic, and auditory symptoms typical of the prodromal or acute uveitic phase and responded well to prompt management. Case Presentation: A young female in her late 20s presented with low-grade fever, severe headache, neck pain, and neck stiffness. She had received symptomatic treatment at another hospital without relief. She was empirically started on intravenous antibiotics and dexamethasone for suspected pyogenic meningitis and was discharged upon symptom relief. However, she returned two days later due to symptom recurrence. Ophthalmic examination revealed decreased visual acuity bilaterally (6/24), sluggish pupil reaction, optic disc edema, and bilateral macular serous detachments. Mild vitritis with anterior chamber cells and iris pigment on the anterior lens capsule was noted in the left eye. Systemic examination was unremarkable, except for fine crepitations in the bilateral lower lung fields. Management: Considering VKH disease, the patient was started on intravenous methylprednisolone pulse therapy (1 gram/day) for 3 days, followed by oral steroids and topical steroid drops for the eyes. She was discharged with oral prednisolone and prednisolone acetate 1% eye drops. At follow-up, her vision improved, and there was resolution of papillitis and serous retinal detachments. Conclusions: VKH is a significant cause of bilateral vision loss. This case of probable VKH syndrome underscores the importance of early recognition and aggressive treatment in achieving a favorable visual prognosis.