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

Treatment of Vogt-Koyanagi-Harada (VKH) Syndrome

High-dose systemic corticosteroids are the first-line treatment for VKH syndrome, followed by immunosuppressive agents for steroid-resistant or dependent cases. 1

Initial Management

First-Line Treatment

• Systemic corticosteroids:
  • Begin with high-dose oral corticosteroids or pulse intravenous corticosteroids
  • For acute phase: Methylprednisolone IV pulse therapy (typically 1g/day for 3 days)
  • Follow with oral prednisone (1-1.5 mg/kg/day)
  • Maintain high-dose therapy for 4-6 weeks
  • Taper slowly over 6+ months to prevent recurrence 2

Key Considerations

• Early and aggressive treatment is crucial to prevent chronic recurrent inflammation
• Inadequate initial treatment or premature tapering increases risk of complications
• Monitor for corticosteroid side effects (elevated intraocular pressure, hyperglycemia, etc.)

Second-Line/Steroid-Sparing Treatments

For cases that are steroid-resistant, steroid-dependent, or with contraindications to steroids:

Immunomodulatory Agents

1. Calcineurin inhibitors 1:

   • Tacrolimus (better tolerated than cyclosporine)
   • Cyclosporine

2. Antimetabolites 1, 3:

   • Mycophenolate mofetil
   • Azathioprine
   • Methotrexate

3. Biologics (for refractory cases) 1:

   • TNF-α inhibitors (e.g., adalimumab) 4
   • Rituximab (for severe refractory cases)

4. Other options 1:

   • Intravenous immunoglobulin
   • Cyclophosphamide (for severe inflammation unresponsive to other agents)

Management Algorithm

1. Acute phase (first 1-2 weeks):

   • High-dose systemic corticosteroids (IV pulse followed by oral)
   • Topical corticosteroids for anterior segment inflammation
   • Monitor visual acuity, OCT, and fluorescein angiography

2. Early follow-up phase (2-8 weeks):

   • Continue high-dose oral corticosteroids
   • Add immunomodulatory therapy if:
     • Inadequate response to corticosteroids after 2 weeks
     • Contraindications to high-dose corticosteroids
     • Signs of steroid dependence

3. Maintenance phase (beyond 8 weeks):

   • Slow taper of corticosteroids over 6+ months
   • Continue immunomodulatory therapy if initiated
   • Regular monitoring for recurrence

4. Long-term follow-up:

   • Monitor for complications (cataracts, glaucoma, subretinal neovascular membranes)
   • Treat associated dry eye aggressively
   • Address extraocular manifestations (auditory, neurological, dermatological)

Monitoring and Follow-up

• Regular follow-up visits based on disease severity and treatment
• Each visit should include:
  • Visual acuity measurement
  • Slit-lamp biomicroscopy
  • Intraocular pressure measurement
  • Dilated fundus examination
  • OCT imaging to monitor subretinal fluid
  • Fluorescein angiography as needed

Complications Management

• Cataracts (occur in ~25% of patients): Surgical management with perioperative immunosuppression 5
• Glaucoma (occurs in ~33% of patients): Medical or surgical management as appropriate 5
• Subretinal neovascular membranes (occur in ~10%): Consider anti-VEGF therapy 5
• Bullous retinal detachment: May require surgical intervention if unresponsive to medical therapy 4

Prognosis

With appropriate treatment, approximately 60% of patients maintain vision of 20/30 or better 5. The major risk factor for poor visual outcomes is chronic recurrent inflammation, emphasizing the importance of early, aggressive, and sustained immunosuppressive therapy.

Pitfalls to Avoid

1. Inadequate initial treatment: Insufficient corticosteroid dosing or premature tapering increases risk of recurrence and complications
2. Delayed treatment initiation: Early intervention is critical for better outcomes
3. Insufficient monitoring: Regular follow-up is essential to detect and manage complications
4. Monotherapy reliance: Refractory cases often require combination therapy
5. Ignoring extraocular manifestations: Comprehensive management should address all systemic aspects of the disease