Treatment of Behçet's Disease with Retinal Vasculitis to Optimize Quality of Life
Primary Treatment Recommendation
All patients with Behçet's disease and retinal vasculitis must receive combination therapy with azathioprine 2.5 mg/kg/day plus systemic corticosteroids as the foundational treatment regimen. 1, 2 This approach has demonstrated sustained long-term benefits including stabilization of visual acuity, reduction in hypopyon uveitis attacks, and prevention of new eye disease development—all critical factors for maintaining quality of life. 1, 3
Initial Treatment Protocol
Corticosteroid Component
- Start with high-dose intravenous methylprednisolone pulses (1 g/day for 3-7 consecutive days) for rapid suppression of acute inflammation. 2, 4
- The addition of IV steroid pulses to conventional therapy results in significantly better visual acuity improvement (0.5 to 0.8 vs 0.6 to 0.7) and fewer flares (1 vs 7 flares in 6 months) compared to conventional therapy alone. 4
- Follow with oral prednisolone 1 mg/kg/day, then taper by 5-10 mg every 10-15 days to reach maintenance dose of 5-10 mg/day. 2
- Continue maintenance corticosteroids for at least 2 years and for at least 12 months after normalization of inflammatory markers. 2
Azathioprine Component
- Initiate azathioprine at 2.5 mg/kg/day—this is the evidence-based dose that decreases hypopyon uveitis attacks (NNT=4) and prevents new eye disease (NNT=2). 1, 3
- Consider gradual dose escalation starting at 50-100 mg daily, increasing to target over 2-4 weeks to minimize gastrointestinal side effects. 3
- Check TPMT activity before starting: use standard dosing for normal activity, reduce to 1-1.5 mg/kg/day for intermediate activity, and avoid entirely for absent TPMT activity. 3
- Continue azathioprine for at least 2 years, with consideration for indefinite maintenance in severe disease. 3
Treatment Escalation for Severe or Refractory Disease
For severe eye disease—defined as >2 lines drop in visual acuity on a 10/10 scale and/or active retinal vasculitis or macular involvement—escalate immediately to either infliximab or cyclosporine A in addition to azathioprine and corticosteroids. 1, 2
Infliximab as Preferred Escalation Agent
- Infliximab demonstrates superior outcomes compared to conventional therapy alone, with significantly reduced relapse rates, longer remission duration, and better visual acuity at 24 months. 5
- Infliximab produces rapid control of inflammation within 1-5 days, with rapid vision improvement in retinal vasculitis patients. 6, 5
- Dosing: 3-5 mg/kg IV on day 1, then every 2 weeks for 6 doses, then maintenance every 4-8 weeks as needed. 6
- Screen for tuberculosis before initiating, particularly important as endemic areas for Behçet's overlap with TB-endemic regions. 2
- Consider drug-free remission after 2 years of successful anti-TNF treatment combined with azathioprine in sight-threatening disease. 2
Cyclosporine A as Alternative
- Cyclosporine A at 2-5 mg/kg/day shows rapid and significant improvement in visual acuity and reduced frequency of ocular attacks. 1, 2
- Critical caveat: Never use cyclosporine A in patients with any central nervous system involvement due to neurotoxicity risk. 2
- Monitor closely for renal dysfunction, hypertension, and nephrotoxicity. 2
Pulse Cyclophosphamide for Aggressive Disease
- For the most aggressive retinal vasculitis, combination of monthly pulse cyclophosphamide (1 g monthly for 6 months, then every 2-3 months) with azathioprine 2-3 mg/kg daily and prednisolone produces excellent long-term outcomes. 7
- This regimen improved mean visual acuity from 3.5 to 4.3, with 44% of eyes improving, and retinal vasculitis scores from 3.0 to 1.4, with 70% of eyes improving over up to 10 years follow-up. 7
- Consider this combination as best treatment choice for retinal vasculitis before opting for biologic agents when infliximab is not available or contraindicated. 7
Quality of Life Considerations
Visual Outcomes
- The primary driver of quality of life in Behçet's retinal vasculitis is preservation of vision, as recurrent inflammatory attacks result in irreversible damage and visual loss. 1
- Suppression of inflammation and prevention of recurrences must be the treatment goals. 1
- Visual acuity improvement is often limited by cataract development, a common complication requiring monitoring and potential surgical intervention. 7
Disease Course Impact
- Behçet's disease follows a relapsing-remitting course that gradually abates over time, but new vascular involvement may occur in up to half of patients. 8
- Retinal vasculitis is the most aggressive ocular lesion and predicts worse systemic outcomes, necessitating aggressive treatment. 7
- Post-thrombotic syndrome from recurrent deep vein thrombosis may result in difficult-to-treat leg ulcers, further impacting quality of life. 2
Monitoring Protocol
- Perform regular ophthalmologic examinations to detect early disease progression. 8
- Monitor complete blood counts and liver function tests monthly for first 3 months, then every 3 months on azathioprine. 3
- Assess clinical symptoms and inflammatory markers (ESR, CRP) regularly. 2
- Perform bone DEXA scanning at 1-2 yearly intervals while on corticosteroids. 2
- Provide calcium and vitamin D supplementation to all patients on corticosteroids. 2
Common Pitfalls to Avoid
- Never use corticosteroids alone in posterior uveitis—they must always be combined with immunosuppressive agents to prevent relapse and achieve steroid-sparing effect. 2
- Never delay escalation to biologics in severe disease—waiting too long risks irreversible vision loss that cannot be recovered even with aggressive treatment. 1, 5
- Never use anticoagulants routinely for venous thrombosis in Behçet's—the primary pathology is vessel wall inflammation requiring immunosuppression, and anticoagulants increase bleeding risk especially with potential coexisting pulmonary arterial aneurysms. 2
- Avoid live vaccines during immunosuppressive treatment. 3