What is the recommended treatment for a patient diagnosed with Vogt-Koyanagi-Harada (VKH) syndrome?

Treatment of Vogt-Koyanagi-Harada Syndrome

The optimal treatment for VKH disease is high-dose systemic corticosteroids combined with mycophenolate mofetil (MMF) initiated immediately at diagnosis, which achieves 20/20 vision in 93.4% of eyes and prevents progression to chronic recurrent inflammation. 1

First-Line Treatment Protocol

Corticosteroid Therapy

• Initiate high-dose systemic corticosteroids immediately upon diagnosis as the cornerstone of therapy 1
• Intravenous methylprednisolone is superior to oral corticosteroids, resulting in fewer relapses (p = 0.026), fewer relapses per patient per month (p < 0.0001), and more relapse-free cases after induction therapy (p = 0.007) 2
• Intravenous corticosteroids also reduce the incidence of sunset glow fundus (33.3% versus 55% with oral steroids), which is associated with worse final visual acuity (p = 0.006) 2
• Continue corticosteroid therapy for at least 6 months with gradual tapering to prevent disease recurrence, which occurs in 43% of patients within the first 3 months when steroids are tapered too rapidly 3

Immunomodulatory Therapy

• Add mycophenolate mofetil (MMF) from the outset of treatment, not as a second-line agent 1
• The combination of MMF with high-dose corticosteroids achieves visual acuity of 20/20 in 93.4% of eyes compared to only 38% with corticosteroids alone 1
• No eyes progressed to chronic recurrent granulomatous uveitis when MMF was used from disease onset 1
• Recurrence of inflammation ≥3 times was significantly reduced from 18% (corticosteroids alone) to 3% (corticosteroids + MMF) 1

Alternative Immunosuppressive Agents

When MMF is contraindicated or not tolerated:

• Methotrexate has comparable efficacy to MMF without evidence of superiority of one over the other (grade C recommendation) 1
• Azathioprine demonstrated control of inflammation in 85.5% of acute cases and 90% of chronic cases with steroid-sparing effect at 4 months 1
• Calcineurin inhibitors (tacrolimus or cyclosporine) are recommended with grade B evidence, though tacrolimus is better tolerated (6% versus 37% adverse events with cyclosporine) 1, 4

Adjunctive Ocular Management

• Apply topical corticosteroids for anterior uveitis and episcleritis 1
• Administer intravitreal anti-VEGF therapy if choroidal neovascularization develops, as this complication is associated with poor visual prognosis 1, 3
• Consider intravitreal triamcinolone for progressive or stubborn cases not responding to systemic therapy 4

Critical Monitoring Requirements

• Perform regular ophthalmologic examinations to detect early disease progression 1
• Monitor intraocular pressure at each visit when using topical corticosteroids 1
• Document visual acuity, slit-lamp findings, and fundoscopic changes at follow-up visits 1
• Screen for systemic adverse effects of immunosuppressive therapy with specialist oversight 1
• Use high-resolution optical coherence tomography (OCT3) to monitor cystoid spaces in the neurosensory layer between inner and outer photoreceptor segments 4

Common Pitfalls to Avoid

• Do not delay immunosuppressive therapy beyond diagnosis - even when initiated at a median of 180 days from symptom onset, immunosuppressive therapy reduces sunset glow fundus incidence and improves visual prognosis 2
• Do not use oral corticosteroids when intravenous formulation is available - oral steroids result in significantly higher relapse rates and worse outcomes 2
• Do not taper corticosteroids rapidly - maintain therapy for at least 6 months with gradual tapering, as rapid reduction causes recurrence in 43% of patients within 3 months 3
• Do not use corticosteroids as monotherapy - the addition of MMF from the outset prevents chronic recurrent inflammation that occurs with steroids alone 1

Expected Outcomes

• Long-term visual prognosis is good with appropriate treatment, with final visual acuity better than 20/30 in 66% of eyes 3
• Poor prognosis is associated with development of choroidal neovascular membranes, chronic uveitis, or sunset glow fundus 2, 3
• Chronic uveitis requiring prolonged corticosteroid therapy (average 48 months) occurs in patients who do not receive adequate initial immunosuppression 3