Yonsei Med J.  2015 Jul;56(4):1158-1162. 10.3349/ymj.2015.56.4.1158.

Clinical Experience of Interferon Alfa-2a Treatment for Refractory Uveitis in Behcet's Disease

• Park JY
• Chung YR
• Lee K
• Song JH
• Lee ES

• KMID: 2366362
• DOI: http://doi.org/10.3349/ymj.2015.56.4.1158

Abstract

Behcet's disease (BD) involves multisystem vasculitis of unknown origin. Ocular manifestations of BD mostly include bilateral panuveitis and retinal vasculitis, which are very challenging to treat. Interferon alfa-2a (IFN) has been recently introduced for treating refractory Behcet uveitis, mainly in Germany and Turkey. Nonetheless, there is so far no consensus about the ideal treatment regimen of IFN for Behcet uveitis. We report our experience of IFN treatment in five Korean BD patients with refractory uveitis. All patients complained of oral ulcers; one patient had a positive pathergy test and 2 showed the presence of HLA-B51. Immunosuppressive agents used prior to IFN treatment included cyclosporine and methotrexate. The IFN treatment was commenced with a dose of 6-9 MIU/day for 7 days, adjusted according to individual ocular manifestations, tapered down to 3 MIU three times in a week, and then discontinued. All patients showed positive response to IFN treatment; 50% of them showed complete response without additional major ocular inflammation during the follow-up period. Other BD symptoms also improved after IFN treatment in most cases. After treatment, the relapse rate and the required dose of oral corticosteroid were decreased in most cases, showing a significant steroid-sparing effect. However, the visual acuity was not improved in most cases due to irreversible macular sequelae. Despite the small sample size of this study, we suggest that, in Korean patients, IFN is an effective treatment modality for BD uveitis as was observed in German and Turkish patients.

Keyword

Behcet's disease; interferon alfa-2a; uveitis

MeSH Terms

Adult
Behcet Syndrome/complications/diagnosis/*drug therapy
Chronic Disease
Cyclosporine/therapeutic use
Female
Humans
Immunosuppressive Agents/administration & dosage
Interferon-alpha/*therapeutic use
Male
Recombinant Proteins/therapeutic use
Recurrence
Remission Induction
Treatment Outcome
Turkey
Uveitis/diagnosis/*drug therapy/etiology
Visual Acuity
Cyclosporine
Immunosuppressive Agents
Interferon-alpha
Recombinant Proteins

Figure

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  Fig. 1 Flow chart for interferon alfa-2a (IFN) dosage. BD, Behçet's disease.

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  Fig. 2 Fundus photography and fluorescein angiography of one patient (Case 4) with Behçet uveitis. (A) Before vascular occlusion (at the 4-month follow-up): retinal vasculitis of the left eye. Fluorescein angiography (FA) shows diffuse leakage of fluorescein dye from the whole retinal vasculature and the optic disc. (B) Retinal vascular occlusion (before interferon alfa-2a treatment, at the 8-month follow-up): branch retinal vein occlusion with intraretinal edema of the left eye. FA shows blocked vascular filling at the inferotemporal venous branch. (C) After interferon alfa-2a treatment (at the 14-month follow-up): tortuous retinal vessels at the inferotemporal area, with improved retinal hemorrhage and edema. FA shows reperfusion; however, narrow inferotemporal venous branch with wide peripheral non-perfusion areas are also noted.

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