What is the treatment for Vogt-Koyanagi-Harada (VKH) disease?

Treatment of Vogt-Koyanagi-Harada Disease

For acute initial-onset VKH disease, initiate high-dose systemic corticosteroids (1-2 mg/kg/day) combined with mycophenolate mofetil (MMF) as first-line immunomodulatory therapy within 2-3 weeks of symptom onset to prevent chronic disease progression and achieve optimal visual outcomes. 1

Immediate First-Line Treatment Protocol

Combination therapy from disease onset is superior to corticosteroid monotherapy:

• Start high-dose systemic corticosteroids (methylprednisolone 1-2 mg/kg/day orally or IV equivalent) immediately upon diagnosis 1, 2
• Add MMF as first-line immunomodulatory therapy simultaneously with corticosteroids, not as sequential therapy 1
• This combination achieves 20/20 visual acuity in 93.4% of eyes versus only 38% with corticosteroids alone 1
• No eyes progress to chronic recurrent granulomatous uveitis when MMF is initiated from the outset 1
• Recurrence of inflammation ≥3 times drops from 18% (steroids alone) to 3% (steroids + MMF) 1

The critical therapeutic window is 2-3 weeks from initial symptom onset - treatment within this period prevents evolution to chronic disease and development of "sunset glow fundus" 3

Alternative First-Line Immunomodulatory Agents

If MMF is contraindicated or unavailable:

• Methotrexate has comparable efficacy to MMF without evidence of superiority (grade C recommendation) 1
• Azathioprine demonstrates inflammation control in 85.5% of acute cases with steroid-sparing effect at 4 months (100 mg/day dose) 1, 2
• Calcineurin inhibitors (tacrolimus or cyclosporine) are recommended with grade B evidence, though tacrolimus is better tolerated (6% vs 37% adverse events with cyclosporine) 1
• Cyclosporine A at 175 mg/day can be used in combination with azathioprine for prolonged therapy 2

Corticosteroid Tapering Strategy

• Begin slow tapering only after achieving complete disease control 2, 4
• Maintain immunosuppressive therapy during and after steroid taper to prevent recurrence 4
• Average time from diagnosis to IMT initiation should be minimized (ideally <2 months) 4

Management of Refractory or Chronic Disease

For patients not responding to first-line therapy or with persistent inflammation:

• TNF-α inhibitors (adalimumab or infliximab) are increasingly used for refractory cases with demonstrated safety and efficacy 2, 4
• Adalimumab is introduced when poor prognostic signs (hypotony, persistent optic disc edema) remain despite conventional immunosuppression 2
• Intravitreal triamcinolone or bevacizumab may be considered for progressive or stubborn cases 5

Note that 50% of patients treated with MMF monotherapy may not achieve disease control, necessitating escalation to biologic agents 4

Adjunctive Ocular Management

For severe ocular inflammation:

• Topical corticosteroids for anterior uveitis and episcleritis 6
• Systemic corticosteroids (prednisolone 1-2 mg/kg) for significant ocular toxicity 6
• Intravitreal anti-VEGF therapy if choroidal neovascularization develops 6

Critical Monitoring Requirements

• 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 6
• Screen for systemic adverse effects of immunosuppressive therapy with specialist oversight 6

Key Clinical Pitfalls to Avoid

Do not use corticosteroid monotherapy - this approach results in significantly worse visual outcomes and higher recurrence rates 1, 4

Do not delay immunomodulatory therapy - waiting beyond the 2-3 week therapeutic window allows progression to chronic disease with irreversible "sunset glow fundus" 1, 3

Do not taper steroids prematurely - maintain adequate immunosuppression for prolonged periods (months) to prevent recurrence 2, 4

Recognize bilateral involvement occurs in 95% of cases - always examine both eyes thoroughly even if symptoms are asymmetric 1