What is the treatment for retinal vasculitis?

Treatment of Retinal Vasculitis

Retinal vasculitis requires immediate initiation of high-dose systemic corticosteroids (prednisolone 1 mg/kg/day, maximum 60 mg/day) as first-line therapy, with early addition of steroid-sparing immunosuppressive agents (mycophenolate mofetil, azathioprine, or methotrexate) for maintenance therapy, while systemic workup proceeds to identify underlying etiology. 1, 2

Initial Management Algorithm

Immediate Treatment Initiation

• Start high-dose oral prednisolone at 1 mg/kg/day (maximum 60 mg/day) without waiting for diagnostic workup completion, as delay risks irreversible vision loss 1, 3
• Maintain initial high dose for at least 4-5 weeks before beginning taper 1, 3
• Therapeutic success with this regimen approaches 60% as monotherapy, improving to 77% with addition of immunosuppressive agents 3

Critical Diagnostic Considerations

• Rule out infectious causes first (tuberculosis, syphilis, toxoplasmosis, viral retinitis) as these require antimicrobial therapy rather than immunosuppression 4
• In patients over 50 years, giant cell arteritis represents an ophthalmologic emergency requiring immediate high-dose corticosteroids to prevent fellow eye involvement 5
• Perform ultra-widefield fluorescein angiography to document extent of vascular involvement, leakage patterns, and peripheral ischemia 6

Steroid-Sparing Immunosuppressive Therapy

First-Line Agents (Grade B Recommendation)

Add immunosuppressive therapy early (within 4-8 weeks) rather than waiting for steroid-related complications, as 50% of patients develop steroid side effects 1, 3

Mycophenolate Mofetil

• Preferred for most cases due to favorable tolerability profile and efficacy in inflammation control 1
• Demonstrates steroid-sparing effect with low discontinuation rates from adverse events 1
• Grade B recommendation for noninfectious uveitis and retinal vasculitis 1

Azathioprine

• Particularly effective for Behçet's disease-associated retinal vasculitis, with 90% inflammation control rate 1, 7
• Reduces relapse rate in all patients when combined with corticosteroids 7
• Achieves steroid dose reduction in 60% of patients 7
• Grade B recommendation, though higher side effect profile than mycophenolate (24% discontinuation rate) 1

Methotrexate

• Demonstrates efficacy in inflammation control and steroid-sparing ability 1
• Grade B recommendation with comparable efficacy to mycophenolate mofetil 1
• Better tolerated than azathioprine with lower discontinuation rates 1

Calcineurin Inhibitors (Alternative Options)

• Tacrolimus or cyclosporine for refractory cases, with tacrolimus better tolerated (6% vs 37% adverse events) 1
• Grade B recommendation, though typically reserved for cases failing antimetabolites 1

Biologic Therapy for Refractory Disease

Adalimumab (Grade A Recommendation)

• Adalimumab is the only biologic with Grade A evidence for noninfectious uveitis, dosed at 80 mg initial dose followed by 40 mg every other week 1, 8
• FDA-approved for adult non-anterior uveitis with demonstrated reduction in uveitic flares and vision loss 8
• Consider when conventional immunosuppression fails or is contraindicated 1

Infliximab (Grade B/C Recommendation)

• Alternative TNF-inhibitor with Grade B/C evidence for refractory cases 1

Management of Macular Edema

For secondary cystoid macular edema complicating retinal vasculitis:

• Intravitreal anti-VEGF agents (bevacizumab, ranibizumab, or aflibercept) represent first-line local therapy with favorable risk-benefit profile 9, 2
• Anti-VEGF therapy addresses macular edema while systemic immunosuppression controls underlying vasculitis 2
• Avoid intravitreal corticosteroids as monotherapy in steroid responders or when systemic inflammation requires control 2

Corticosteroid Tapering Protocol

• Do not use alternate-day therapy, as this increases relapse risk 1
• Target dose of 10-15 mg/day by 3 months if inflammation controlled 1
• Duration of therapy often extends several years; some patients cannot fully discontinue 1
• Provide bone protection therapy (bisphosphonates, calcium, vitamin D) for all patients on long-term corticosteroids 1

Common Pitfalls to Avoid

Inadequate Initial Steroid Dosing

• Most common reason for apparent treatment failure is starting with insufficient corticosteroid dose (<1 mg/kg/day) or tapering too rapidly (before 5 weeks) 3

Delayed Immunosuppressive Addition

• Waiting for steroid complications before adding steroid-sparing agents increases cumulative steroid exposure and morbidity 3, 10

Misdiagnosis of Underlying Etiology

• Systemic lupus erythematosus-associated retinal vasculitis may require hydroxychloroquine in addition to immunosuppression 10
• Sarcoidosis-associated retinal vasculitis presents with characteristic peripheral granulomas and requires systemic evaluation 1

Ignoring Peripheral Ischemia

• Persistent vascular filling defects on fluorescein angiography may not resolve despite inflammation control and require monitoring for neovascular complications 6

Disease-Specific Considerations

Behçet's Disease

• Azathioprine combined with systemic corticosteroids is specifically indicated, with vessel wall inflammation (not thrombophilia) as primary pathology 1, 5, 7

Vogt-Koyanagi-Harada Disease

• Requires aggressive initial corticosteroid therapy with early immunosuppressive addition (mycophenolate mofetil or methotrexate) 1
• Bilateral presentation in 95% of cases with characteristic OCT findings 1

Drug-Induced Vasculitis (Brolucizumab)

• Requires prompt systemic and local steroid therapy (oral prednisolone 30 mg/day plus subtenon triamcinolone) when anti-VEGF-associated retinal vasculitis occurs 6
• Discontinue offending agent permanently 6