Background/Purpose: Ocular lesions of Behcet’s Disease (BD), need aggressive treatment to prevent severe loss of vision or blindness. Cytotoxic drugs are the main therapeutic agents and the first line treatment for it. Retinal vasculitis is the most aggressive lesion of ocular manifestations. It has the worse outcome. We present here the outcome with a combination of pulse cyclophosphamide, azathioprine, and prednisolone, on long-term usage up to 10 years on 291 patients (17286 eyes-months of follow-up).

Methods: Cyclophosphamide was used as one gram as monthly pulse for 6 months and then every 2 to 3 months as necessary. Azathioprine was used as 2 to 3 mg/kg/weight/daily. Prednisolone was associated as 0.5 mg/kg/ daily. Upon the suppression of the inflammatory reaction, prednisolone was tapered gradually. Inclusion Criteria: 1- Fulfilling the International criteria, the ICBD. 2- Having active posterior uveitis (PU) and/or retinal vasculitis (RV). Visual acuity (VA): was calculated by the Snellen chart on a scale of 10 (best vision 10/10). An activity index was calculated for PU and RV. A Total Adjusted Disease Activity Index (TADAI) was calculated for both eyes taking in account all parameters. Results were assessed at 3, 6, 9 months, then at 1, 1.5, 2, 2.5, 3, 4, 5, 6, 7, 8, 9, and 10 years.  

Results: The mean improvement for Visual Acuity (p value) was: 0.9 (<0.001), 0.9 (<0.001), 0.9 (<0.001), 1.0 (<0.001), 0.8 (<0.001), 0.9 (<0.001), 0.9 (<0.001), 0.9 (<0.001), 1.1 (0.005), 0.1 (0.9), -0.5 (0.3), -0.5 (0.35), +2.0 (0.2), +2.9 (0.2), +0.3 (0.9). The mean improvement for Posterior Uveitis was: 1 (<0.001), 1.2 (<0.001), 1.5 (<0.001), 1.4 (<0.001), 1.4 (<0.001), 1.6 (<0.001), 1.6 (<0.001), 1.7 (<0.001), 1.9 (<0.001), 1.9 (<0.001), 1.9 (<0.001), 2 (<0.001), 2 (0.01), 2.8 (0.08), 1 (0.9). The mean improvement for Retinal Vasculitis was: 1.1 (<0.001), 1.7 (<0.001), 1.6 (<0.001), 1.6 (<0.001), 1.8 (<0.001), 1.7 (<0.001), 1.9 (<0.001), 1.8 (<0.001), 2.1 (<0.001), 2.2 (<0.001), 2.2 (<0.001), 1.8 (0.004), 6 (0.11), 4 (0), 2 (0). The mean improvement for TADAI was: 9.1 (<0.001), 11.7 (<0.001), 12.7 (<0.001), 11.9 (<0.001), 13 (<0.001), 13.8 (<0.001), 14.2 (<0.001), 14.6 (<0.001), 13.5 (<0.001), 16.3 (<0.001), 19.6 (<0.001), 17.1 (<0.001), 16.2 (0.6), 5.4 (0.2), 4 (0.6). Overall results (from baseline to the last evaluation): The mean VA improvement was 0.8 (<001), PU 1.4 (<0.001), RV 1.6 (<0.001), and TADAI 11 (<0.001). VA improved in 45.4% of eyes, PU in 75.6% of eyes, RV in 71.5% of eyes, and TADAI in 74.9% of patients. VA aggravated in 33% of eyes, PU in 14% of eyes, RV in 16.9% of eyes, and TADAI in 18.9% of patients. The remaining kept their baseline values. 

Conclusion: All parameters improved significantly. The improvement in VA was the least. It was mainly due to cataract. The non-significance of p values in the last years of follow-up was due to the low number of patients. Combination of pulse cyclophosphamide and azathioprine is the best treatment choice for retinal vasculitis before opting for biologic agents.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/behcets-disease-combination-of-pulse-cyclophosphamide-azathioprine-and-prednisolone-for-the-treatment-of-retinal-vasculitis-longitudinal-study-on-10-years/