Combination of pulse cyclophosphamide and azathioprine in ocular manifestations of Behcet’s disease: longitudinal study of up to 10 years


Abstract: Aim: 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. Retinal vasculitis is the most aggressive lesion of ocular manifestations and predicts a worse systemic outcome. We present here the outcome with a combination of pulse cyclophosphamide, azathioprine and prednisolone, on long-term usage, up to 10 years, on 295 patients (18 493 eye-months of follow-up). Methods: Cyclophosphamide was used as a 1-g monthly pulse for 6 months and then every 2–3 months as necessary. Azathioprine was used at 2–3 mg/kg daily. Prednisolone was initiated at 0.5 mg/kg daily. Upon the suppression of the inflammatory reaction, prednisolone was tapered gradually. Patients fulfilled the International Criteria Behcet’s Disease (ICBD) and had active posterior uveitis (PU) and/or retinal vasculitis (RV). Visual acuity (VA), PU, RV and TADAI (Total Adjusted Disease Activity Index) were calculated. Results: Overall results: mean VA improved from 3.5 to 4.3 (P < 0.0001), 44% of eyes improved (95% CI = 40–50). Mean PU improved from 2.1 to 0.8 (P < 0.0001), 73% of eyes improved (95% CI = 69–78). Mean RV improved from 3.0 to 1.4 P < 0.0001), 70% of eyes improved (95% CI = 65–74). Mean TADAI improved from 29 to 18 (P < 0.0001), 72% of patients improved (95% CI = 66–77). The details of the longitudinal studies are given in the main article. Conclusion: All parameters significantly improved. VA improvement was the least, mainly due to cataracts. This combination is the best treatment choice for retinal vasculitis before opting for biologic agents. Key words: Behcet’s disease, cytotoxic drugs, longitudinal study, ocular lesions.