Systemic Immunomodulatory Therapy in Uveitis Related to Behçet's Disease: a 10-year Profile

Abstract

Purpose: Behçet's disease (BD) is a systemic vasculitis that is often accompanied by intraocular inflammation. This study aims to determine the efficacy and safety of systemic immunomodulatory therapy (IMT) in Behçet's disease and the optimal timing for its discontinuation in patients in remission. Patients and methods: A retrospective single-center longitudinal study was performed at the Centro Hospitalar Universitário São João (Porto, Portugal). Thirty-eight records of patients with uveitis related to Behçet's disease and on immunomodulatory therapy were analyzed for demographic data, characteristics of their uveitis, treatment period, pattern of relapses, and discontinuation outcomes. The statistical analyses were done with IBM SPSS® software. Results: The mean follow-up duration was 122.5 ± 62.6 months. Anterior uveitis was the most common manifestation (36.8%), followed by retinal vasculitis (31.6%) and panuveitis (13.2%). Azathioprine (36.8%) and cyclosporine (28.9%) were the most used immunomodulatory agents. The median treatment duration was 63.5 months, significantly reducing relapse rates from 2 ± 2.0 to 1 ± 1.2 per year (p < 0.001). Biologic therapies showed a slight advantage in reducing relapses (p = 0.045). Among 16 patients (42.1%) who discontinued treatment, 30.8% experienced relapse after a median of 13 months. Eight out of twelve patients who stopped treatment and were treated for more than 4 years did not relapse. Patients treated for more than 6 years had no relapses. Conclusion: IMT effectively controls ocular inflammation in BD uveitis, significantly reducing relapse frequency. Azathioprine and cyclosporine remain first-line therapies. Discontinuing IMT after 4-6 years of sustained remission appears to be a safe strategy, particularly after 6 years.