What is the treatment for retinal vasculitis?

Treatment of Retinal Vasculitis

The treatment of retinal vasculitis requires early initiation of high-dose systemic corticosteroids as first-line therapy, with consideration of immunosuppressive agents as adjunctive therapy based on etiology, severity, and response to initial treatment. 1, 2

Initial Assessment and Classification

• Determine if retinal vasculitis is:

  • Isolated ocular disease or part of systemic condition
  • Associated with infectious vs. non-infectious etiology
  • Acute (within 24 hours) or chronic
  • In patients over 50, evaluate for giant cell arteritis (GCA) 1, 2

• Key diagnostic workup:

  • Targeted serologies based on suspected etiology:
    • ANCA for autoimmune vasculitis
    • Anti-SSA/Ro for Sjögren's syndrome
    • Specific tests for infectious causes (Bartonella, Francisella) 2
  • In patients >50 years: ESR, CRP to rule out GCA 1, 2
  • Fluorescein angiography to confirm diagnosis and assess severity 3

Treatment Algorithm

1. Giant Cell Arteritis-Associated Retinal Vasculitis (patients >50)

• Immediate high-dose systemic corticosteroids (1 mg/kg/day prednisolone, max 60 mg/day) 1
• Maintain high dose for one month, then taper gradually 1
• Do not use alternate-day therapy (increases relapse risk) 1
• Consider IV methylprednisolone for patients with early visual symptoms 1
• Bone protection therapy should be provided 1

2. Non-Arteritic Retinal Vasculitis

First-Line Treatment:

• High-dose systemic corticosteroids (≥1 mg/kg prednisolone) 4
• Maintain high dose (≥40 mg prednisolone) for at least 5 weeks 4
• Success rate with this regimen: approximately 60%, improving to 77% with addition of immunosuppressives 4

Second-Line/Adjunctive Therapy:

• Immunosuppressive agents should be considered when:

  • Inadequate response to corticosteroids
  • Corticosteroid-related adverse events (occur in ~50% of patients) 4
  • Need for long-term therapy 1

• Recommended immunosuppressive options:

  • Mycophenolate mofetil (Grade B recommendation) 1
  • Methotrexate (Grade B recommendation) 1
  • Azathioprine (Grade B recommendation) 1
  • Calcineurin inhibitors (tacrolimus, cyclosporine) (Grade B recommendation) 1
  • Adalimumab for non-infectious uveitis (Grade A recommendation) 1

3. Retinal Vasculitis with Arterial Occlusion

• For acute retinal artery occlusion (symptoms within 24 hours):
  • Urgent referral to stroke center due to high risk of concurrent cerebrovascular accident 1, 2
  • Consider hyperbaric oxygen therapy (100% oxygen over 9 hours) 1

4. Disease-Specific Approaches

• Behçet's Disease with retinal vasculitis:

  • Corticosteroid pulse therapy shows better results than oral steroids 5
  • Combination therapy with cyclophosphamide (200 mg/week IV) or cyclosporine (3.5 mg/kg/day) plus prednisolone (10-15 mg/day) for long-term control 5

• Systemic Lupus Erythematosus with retinal vasculitis:

  • Corticosteroid-sparing strategies with cytotoxic agents are effective 6
  • Options include hydroxychloroquine alone or in combination with corticosteroids or cytotoxic agents 6

Monitoring and Follow-up

• Regular ophthalmologic follow-up to monitor for:

  • Treatment response
  • Development of retinal or iris neovascularization
  • Neovascular glaucoma 1, 2

• If neovascularization develops:

  • Panretinal photocoagulation (PRP) is recommended 1, 2
  • Consider anti-VEGF agents when hemorrhage is present 1

Common Pitfalls and Caveats

1. Delayed treatment: Failure to initiate adequate corticosteroid dose early is the most common reason for treatment failure 4

2. Inadequate initial dosing: Starting with too low a dose of corticosteroids reduces effectiveness 4

3. Failure to screen for GCA in patients over 50, which requires immediate treatment to prevent vision loss in the fellow eye 1, 2

4. Overlooking systemic associations: Retinal vasculitis may be the presenting sign of a systemic disease requiring specific treatment 7

5. Neglecting stroke risk: Failure to refer patients with acute retinal artery occlusion for urgent stroke evaluation 1, 2