What is the common course of treatment for Behcet's disease with retinal vasculitis?

Treatment of Behçet's Disease with Retinal Vasculitis

The standard treatment for Behçet's disease with retinal vasculitis includes a combination of azathioprine (2.5 mg/kg/day) and systemic corticosteroids as first-line therapy, with escalation to either cyclosporine A or infliximab for severe or refractory cases. 1

Initial Treatment Approach

• Any patient with Behçet's disease and inflammatory eye disease affecting the posterior segment should be on a treatment regimen that includes azathioprine (2.5 mg/kg/day) and systemic corticosteroids 1
• Azathioprine has been shown to decrease hypopyon uveitis attacks, stabilize visual acuity, and decrease the development of new eye disease with long-term benefits 1
• Systemic corticosteroids are typically started at high doses (1 mg/kg/day of prednisolone) for rapid suppression of inflammation during acute attacks 1, 2
• Local and systemic corticosteroids are generally used during acute attacks, but should never be used alone in patients with posterior uveitis 1, 2

Treatment for Severe or Refractory Disease

• For severe eye disease (defined as >2 lines of drop in visual acuity on a 10/10 scale and/or retinal vasculitis or macular involvement), treatment should be escalated to either cyclosporine A or infliximab in combination with azathioprine and corticosteroids 1
• Cyclosporine A (2-5 mg/kg/day) has demonstrated rapid and significant improvement in visual acuity and reduced frequency and severity of ocular attacks in three RCTs, though renal dysfunction, hypertension, and nephrotoxicity are concerns 1
• Infliximab has shown promising results for refractory eye disease, particularly in combination with other immunosuppressives, with rapid response times (1-5 days) 1, 3
• Interferon-alpha, alone or in combination with corticosteroids, appears to be a second-choice option for eye disease with sustained responses and high remission rates 1, 4

Specific Treatment for Retinal Vasculitis

• Retinal vasculitis is the most aggressive ocular manifestation of Behçet's disease and predicts worse outcomes, requiring particularly aggressive treatment 5
• A combination of pulse cyclophosphamide (1g monthly for 6 months, then every 2-3 months as needed), azathioprine (2-3 mg/kg daily), and prednisolone (starting at 0.5 mg/kg daily) has shown significant improvement in visual acuity, posterior uveitis, and retinal vasculitis in long-term studies 5
• Alternatively, a combination of cyclophosphamide (200 mg/week intravenously) with prednisolone (10-15 mg/day) or cyclosporine (3.5 mg/kg/day) with prednisolone (10-15 mg/day) has been effective for long-term control of retinal vasculitis 6

Monitoring and Follow-up

• Regular ophthalmologic examinations are essential to detect early signs of disease progression 7
• Monitor for azathioprine side effects with liver function tests monthly for the first 3 months, then every 3 months 8
• Check thiopurine methyltransferase (TPMT) activity prior to initiating azathioprine therapy to guide dosing and reduce risk of severe myelosuppression 8
• Assess clinical symptoms and inflammatory markers (ESR, CRP) regularly to evaluate treatment response 2

Prognosis and Disease Course

• Behçet's disease follows a relapsing and remitting course that gradually abates with time 7
• The prognosis of anterior uveitis is generally good, but patients with posterior segment involvement (including retinal vasculitis) tend to have some degree of visual loss even with adequate treatment 9
• Ocular lesions may improve with immunosuppressive therapy but are usually not fully reversible and generally progress over time without adequate treatment 9

Common Pitfalls and Considerations

• Avoid cyclosporine A in patients with central nervous system involvement due to potential neurotoxicity 2
• Consider drug-free, long-term remission after withdrawal of successful anti-TNF treatment combined with azathioprine given for 2 years in a proportion of patients with sight-threatening ocular disease 1
• Due caution for tuberculosis is important when using infliximab, especially as endemic areas for Behçet's disease are also endemic for tuberculosis 1