Introduction: Vogt–Koyanagi–Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by bilateral granulomatous panuveitis often associated with extraocular manifestations. This article provides an in-depth exploration of VKH disease, including its etiology, clinical features, diagnostic approach, management strategies, and prognosis.

Etiology: VKH disease is believed to result from an autoimmune-mediated attack on melanocytes in various tissues, including the uvea, meninges, skin, and inner ear. Genetic predisposition, environmental triggers, and viral infections are thought to contribute to disease pathogenesis, although the exact etiology remains incompletely understood.

Clinical Features:

• Ocular Manifestations: VKH disease typically presents with bilateral, diffuse choroiditis, uveitis, and serous retinal detachments. Patients may experience ocular pain, photophobia, blurred vision, and posterior synechiae. Other findings include disc edema, exudative retinal detachments, and sunset glow fundus.
• Extraocular Manifestations: VKH disease can involve various organ systems, leading to manifestations such as meningismus, headache, tinnitus, vitiligo, alopecia, and auditory disturbances. Cutaneous findings may include poliosis and depigmented patches.

Diagnostic Approach: Diagnosis of VKH disease relies on a combination of clinical findings, laboratory tests, and imaging studies:

• Ophthalmic examination with dilated fundoscopy to assess for characteristic uveitis, choroiditis, and serous retinal detachments.
• Ancillary tests such as optical coherence tomography (OCT), fluorescein angiography, and indocyanine green angiography to evaluate retinal and choroidal involvement.
• Laboratory investigations including complete blood count, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), and HLA typing to assess for systemic inflammation and genetic predisposition.

Management Strategies: Treatment of VKH disease aims to suppress ocular inflammation, prevent recurrences, and preserve vision:

• Corticosteroids are the mainstay of therapy, with high-dose systemic corticosteroids initially used to induce remission, followed by a gradual tapering regimen. Topical corticosteroids and periocular injections may also be employed for anterior segment inflammation.
• Immunomodulatory agents such as azathioprine, methotrexate, mycophenolate mofetil, or biologic agents like infliximab or adalimumab may be considered in cases of steroid dependence or intolerance.

Prognosis: The prognosis of VKH disease varies depending on several factors, including the severity of ocular and systemic involvement, the promptness of treatment initiation, and the response to therapy. Early diagnosis and aggressive management can lead to favorable visual outcomes and minimize disease-related complications. However, delayed or inadequate treatment may result in permanent vision loss, chronic uveitis, and significant morbidity.

Reference Sites:

1. American Uveitis Society – https://uveitissociety.org/
2. National Eye Institute (NEI) – https://www.nei.nih.gov/
3. PubMed Central (PMC) – [Link to relevant research articles and clinical guidelines]

By providing a comprehensive overview of VKH disease, this article aims to enhance awareness among ophthalmologists and promote early recognition and appropriate management of this complex autoimmune disorder. Continued research efforts are essential to further elucidate disease pathogenesis and optimize treatment strategies for improved patient outcomes.