Abstract
Purpose:
To retrospectively assess the frequency of ocular relapse and the possibility of long-term remission in patients who were treated with interferon (IFN) alpha for severe uveitis associated with Behçet's disease (BD)
Methods:
All patients with severe uveitis associated with BD and referred to Pitié-Salpêtrière Hospital, France, between June 1994 and January 2010 and treated by interferon alpha, whatever the treatment duration, were included in our retrospective cohort study. All patients were treated with initial dosage of IFN alpha 3 million IU thrice a week, increased to 6 million IU thrice a week in case of relapse or discontinued in case of stable clinical examination. All patients had active severe uveitis : refractory to at least one conventional immunosuppressive drug and/or requiring high doses oral corticosteroids (> 10mg per day). All patients whose compliance with medication could not be established were excluded.The main assessment criterion was the number of relapses per patient per year before and after initiation of IFN and after discontinuation of IFN. Best visual acuity, anterior segment inflammation, vitritis, presence of vasculitis, macular edema, papillitis and retinitis were assessed at initiation and at 2, 4 and 9 years after the initiation of IFN.
Results:
Of 36 patients (67 eyes), 31 (86.1%) responded to IFN. For responders, the mean relapse per person per year decreased significantly from 1.39 to 0.0496 (p = 1.82*10^-10) during treatment period. After cessation of IFN, possible for 21 patients, the mean annual incidence of relapse remained at 0.057 relapses per person per year. The mean period from the initiation of IFN to the date of the last follow-up consultation was 8.19 years (range, 6 to 216 months).Thirty three, 25 and 18 patients were followed respectively at 2, 4 and 9 years. Visual acuity was stable or improved for 90.3% of all patients. For the group "9 years", anterior segment inflammation and vitreous haze were reduced during the first year. The prevalence of macular edema and the prevalence of retinitis and papilledema plummeted to zero at 4 years and 1 year after IFN initiation.
Conclusions:
The incidence of relapses in severe uveitis associated with Behçet's disease decreases under IFN alpha treatment. This treatment also seems to permit a long-term remission even after discontinuation. It is an efficient treatment for vasculitis, cystoid macular edema and papilledema.