What is the common treatment approach for a patient with Behçet's disease presenting with retinal vasculitis?

Treatment and Disease Progression in Behçet's Disease with Retinal Vasculitis

Any patient with Behçet's disease and retinal vasculitis should receive a treatment regimen that includes azathioprine (2.5 mg/kg/day) and systemic corticosteroids, with escalation to either cyclosporine A or infliximab for severe cases. 1

Initial Treatment Approach

• Retinal vasculitis in Behçet's disease follows a remitting and relapsing course, with recurrent inflammatory attacks leading to irreversible damage and visual loss if not properly treated 2
• The primary treatment goal should be suppression of inflammation and prevention of recurrent ocular attacks to preserve vision 2, 1
• Azathioprine (2.5 mg/kg/day) has been shown to decrease hypopyon uveitis attacks, stabilize visual acuity, and decrease the development of new eye disease with long-term benefits 2, 1
• Systemic corticosteroids are typically started at high doses (1 mg/kg/day of prednisolone) for rapid suppression of inflammation during acute attacks 1
• Corticosteroids should never be used alone in patients with posterior uveitis/retinal vasculitis due to potential side effects including cataracts and glaucoma 2, 1

Management of Severe or Refractory Disease

• For severe eye disease (defined as >2 lines drop in visual acuity on a 10/10 scale and/or retinal vasculitis or macular involvement), treatment should be escalated 2, 1
• Either cyclosporine A (2-5 mg/kg/day) or infliximab should be added to the azathioprine and corticosteroids regimen 2, 1
• Infliximab has shown promising results for refractory eye disease with rapid response times (1-5 days) and significantly reduced number of relapses compared to conventional therapy 1, 3
• Interferon-alpha, alone or in combination with corticosteroids, is another option for eye disease with sustained responses and high remission rates 1, 4
• A combination of pulse cyclophosphamide with azathioprine and prednisolone has shown significant improvement in visual acuity, posterior uveitis, and retinal vasculitis in long-term studies 5

Disease Progression and Monitoring

• Without proper treatment, retinal vasculitis typically progresses with recurrent inflammatory attacks leading to irreversible damage and eventual visual loss 2, 6
• Regular ophthalmologic examinations are essential for monitoring disease activity and treatment response 1, 7
• Young men with early disease onset have a higher risk of severe disease course and may benefit from early aggressive systemic immunosuppression 1, 7
• Ocular lesions may improve with immunosuppressive therapy but are usually not fully reversible and generally progress over time without adequate treatment 6
• The visual prognosis is generally poor for patients with posterior segment involvement (including retinal vasculitis) compared to those with only anterior uveitis 6

Important Considerations and Pitfalls

• Cyclosporine A should not be used in patients with central nervous system involvement due to potential neurotoxicity 1, 8
• Screening for tuberculosis is important when using infliximab, especially as endemic areas for Behçet's disease are also endemic for tuberculosis 1
• Combination therapy with multiple immunosuppressants may lead to increased risk of myelosuppression, requiring close monitoring of blood counts 4
• Consider drug-free, long-term remission after withdrawal of successful anti-TNF treatment combined with azathioprine given for 2 years in patients with well-controlled disease 1
• Retinal vasculitis is considered the most aggressive ocular manifestation and predicts a worse systemic outcome, requiring more aggressive treatment 5