Background
Behcet’s disease (BD) is a chronic relapsing multisystem vasculitis mainly characterized by recurrent oral ulceration, genital ulceration, ocular lesions, and skin lesions [
1]. Ocular involvement is one of the most serious complication of BD, as repeated attacks of uveitis may result in blindness [
2].
Corticosteroid treatment is the mainstay in the management of acute uveitic attacks, and immunosuppressive agents such as cyclosporine and azathioprine are usually effective in long-term management [
3,
4]. Interferon alpha-2a (IFNα2a) has been reported to be effective and safe in refractory cases, although the optimal regimen has not yet been established [
5‐
13]. In this study, we aimed to evaluate the efficacy of IFNα2a in Korean patients with Behcet uveitis refractory to immunosuppressive agents.
Discussion
In this study, we evaluated the efficacy of IFNα2a in patients with Behcet uveitis refractory to immunosuppressive agents. Most patients had good responses to IFNα2a. IFNα2a therapy was maintained in these patients. There were no uveitis attacks during the treatment period in the 4 patients who were responsive to IFNα2a therapy. Visual acuity improved in all patients.
Corticosteroid is the main treatment option for acute attacks of Behcet uveitis. However, its long-term use is limited because of adverse effects. Cyclosporine and azathioprine have been effectively used in Behcet uveitis alone or combined with other immunosuppressants in severe cases [
3,
4]. There are, however, some patients who are refractory to immunosuppressive agents, and biological therapies including anti-tumor necrosis factor antibody, anti-interleukin, or interferon can be considered in such cases [
16,
17]. Recently, IFNα2a has been reported to be effective for the treatment of refractory Behcet uveitis [
5,
7‐
13,
18,
19].
There is no consensus on the dose and protocol of IFNα2a therapy for Behcet uveitis. In this study, we used a lower-dose regimen of 3 × 10
6 IU of IFNα2a 3 times per week during the treatment period. The rate of treatment response in our series was 80%, which is similar to that in previous reports using higher doses of IFNα2a [
13,
19]. A lower-dose regimen may be associated with fewer treatment-related complications. There were no severe adverse effects in the present study. In contrast, patients with leukopenia or thrombocytopenia have been reported in previous studies using higher doses of IFNα2a [
18,
20]. Four responsive patients could not discontinue IFNα2a therapy in this study, which may also have been associated with the lower dose of the regimen. By comparison, 25–50% of patients may discontinue IFNα2a treatment with higher-dose regimens [
11,
13].
The relapse rate of uveitis attacks significantly decreased from 2.16 ± 1.08 to 0.40 ± 0.89 after IFNα2a therapy. In the 4 patients who had responses to IFNα2a therapy, there were no uveitis attacks during the treatment period. The efficacy of IFNα2a therapy in terms of uveitis relapse was comparable to recent reports [
13,
19]. We confirmed that treatment response without uveitis relapse may be achieved mostly with low-dose continuous IFNα2a therapy in Korean patients.
As refractory Behcet uveitis cases are rare, the major limitations of this study are its retrospective design and the small number of patients. We were, however, able to confirm the efficacy of IFNα2a therapy in a uniform low-dose regimen. Questions regarding the optimal dosage, treatment duration, and treatment protocol of IFNα2a therapy still remain unanswered. A prospective study would be necessary not only to determine the most effective and safest protocol, but also to compare the efficacy of IFNα2a with new biological agents currently under study.
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