Vogt-Koyanagi-Harada syndrome

Area of Science:

• Ophthalmology
• Immunology
• Genetics

Background:

• Vogt-Koyanagi-Harada (VKH) syndrome is a rare, bilateral, diffuse granulomatous uveitis.
• It presents with ocular inflammation and extraocular manifestations including neurological and auditory signs, poliosis, vitiligo, and alopecia.
• The syndrome is thought to involve an autoimmune response targeting melanocytes, influenced by genetic factors.

Purpose of the Study:

• To review the histopathology, diagnosis, management, and prognosis of Vogt-Koyanagi-Harada syndrome.
• To highlight the complications leading to visual loss and identify risk factors.
• To emphasize the role of corticosteroid therapy in managing VKH syndrome and its sequelae.

Main Methods:

• Histopathologic examination revealing non-necrotizing granulomatous panuveitis and Dalen-Fuchs' nodules.
• Diagnostic adjuncts include fluorescein angiography, lumbar puncture, and echography.
• Treatment strategies involve systemic corticosteroids, cyclosporine, or cytotoxic agents.

Main Results:

• VKH syndrome exhibits characteristic histopathological findings.
• Common complications leading to visual impairment include cataracts (25%), glaucoma (33%), and subretinal neovascular membranes (SRNVMs) (10%).
• Chronic, recurrent intraocular inflammation resistant to therapy is a major risk factor for these complications.

Conclusions:

• Aggressive initial high-dose corticosteroid therapy combined with prolonged treatment may minimize sight-threatening complications.
• Effective management of VKH syndrome requires addressing both ocular and extraocular manifestations.
• Optimizing treatment strategies can improve the long-term visual prognosis for patients with VKH syndrome.