What is the treatment for retinal vasculitis?

Treatment of Retinal Vasculitis

High-dose systemic corticosteroids (1 mg/kg/day prednisolone, maximum 60 mg/day) are the first-line treatment for retinal vasculitis, with immunosuppressive agents added for steroid-sparing effects or in refractory cases. 1

Initial Treatment Approach

Corticosteroid Therapy

• Begin with high-dose oral prednisolone at 1 mg/kg/day (maximum 60 mg/day)
• Maintain high-dose therapy for at least one month before gradual tapering 1
• Consider IV methylprednisolone for patients with severe disease or early visual symptoms 1
• Inadequate initial dosing is the most common reason for treatment failure 2

Monitoring Response

• Regular ophthalmologic follow-up to assess:
  • Improvement in visual acuity
  • Resolution of retinal inflammation
  • Development of complications (neovascularization, macular edema)

Second-Line and Adjunctive Therapy

Immunosuppressive Agents

Add immunosuppressive agents when:

• Inadequate response to corticosteroids
• Corticosteroid-related adverse events
• Need for long-term therapy 1

Recommended Immunosuppressives (in order of evidence strength):

1. Azathioprine - Strong evidence for efficacy in Behçet's disease-related retinal vasculitis 3
2. Mycophenolate mofetil - Grade B recommendation for non-infectious uveitis 3
3. Methotrexate - Grade B recommendation for non-infectious uveitis 3
4. Calcineurin inhibitors (tacrolimus, cyclosporine) - Grade B recommendation 3
5. Biologics:
   • Adalimumab - Grade A recommendation for non-infectious uveitis 3
   • Infliximab - Grade B/C recommendation 3
   • Interferon alfa-2a - Supported for non-anterior non-infectious uveitis 3

Specific Considerations for Behçet's Disease

• Patients with Behçet's disease and posterior segment involvement should receive azathioprine and systemic corticosteroids 3
• For severe eye disease (>2 lines drop in visual acuity or retinal vasculitis), add either:
  • Cyclosporine A or infliximab in combination with azathioprine and corticosteroids, or
  • Interferon-α with or without corticosteroids 3

Management of Complications

Neovascularization

• If retinal or iris neovascularization develops, perform panretinal photocoagulation (PRP) 3
• Consider anti-VEGF agents as adjunctive therapy when complete PRP is insufficient to control angiogenesis 3

Macular Edema

• Anti-VEGF agents (ranibizumab, aflibercept, or off-label bevacizumab) are first-line 3
• Intravitreal corticosteroids are second-line due to side effects (glaucoma, cataracts) 3

Treatment Algorithm Based on Etiology

1. Non-infectious isolated retinal vasculitis:

   • High-dose systemic corticosteroids → Add immunosuppressive if needed

2. Behçet's disease with retinal vasculitis:

   • Azathioprine + corticosteroids → Add cyclosporine A/infliximab/interferon-α if severe

3. Systemic vasculitis with retinal involvement:

   • High-dose corticosteroids + appropriate immunosuppressive based on underlying condition

4. Retinal vasculitis with vascular occlusion:

   • Treat underlying inflammation + consider anti-VEGF for macular edema
   • PRP for neovascularization

Common Pitfalls to Avoid

• Inadequate initial corticosteroid dosing - Most common reason for treatment failure 1, 2
• Failure to screen for systemic associations - May lead to delayed diagnosis of underlying disease 1
• Neglecting to monitor for ocular complications - Regular follow-up is essential to detect neovascularization and macular edema 1
• Premature discontinuation of therapy - Inflammation control requires adequate duration of treatment

The therapeutic success rate with high-dose systemic steroids alone is approximately 60%, improving to 77% with the addition of immunosuppressive agents 2. While side effects of steroids are common (50% of cases), they are therapeutically significant in only a minority of patients 2.

I hope this helps with your treatment approach to retinal vasculitis.