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Editor,—One of the main problems in ocular Behçet's disease (BD) is severe posterior uveitis with retinal vessel occlusion and secondary ischaemic changes leading to retinal neovascularisation and to bad prognosis despite immunosuppression.
Previously we have shown the efficacy of interferon alfa (IFNα)-2a in posterior uveitis and especially in retinal vasculitis with reopening of occluded vessels.1 Now we demonstrate the antiangiogenic effect of IFNα-2a in one BD patient with retinal neovascularisations. The IFNα-2a treatment has resulted in complete remission of the retinal neovascularisations without laser coagulation of non-perfusion areas, which would have been the standard therapy.
A 27 year old man with recurrent oral aphtosis, pustular skin lesions, epididymitis, arthritis (elbow, sacroiliac joint), and retinal vasculitis of the left eye was diagnosed in October 1998 as having Behçet's disease (BD), according to the criteria of the international study group.1 At this time therapy with cyclosporin A (3 mg/kg body weight) was initiated. Until June 1999 he had had no relapses. One month later the visual acuity in the right eye decreased to 20/100, but was stable in the left eye (20/600). Biomicroscopically there was no inflammation of the anterior chamber. Funduscopy of both eyes revealed vitreous infiltration and a macular oedema with gliotic changes and central sanguination. In addition, optic disc neovascularisation in the right eye (Fig 1) and optic disc oedema in the left eye were present. Peripheral vessel leakage as a sign of active vasculitis was shown by fluorescein angiography. At the same time oral aphtosis and pustular skin lesions recurred. Owing to active retinal vasculitis with marked decrease of visual acuity we stopped the cyclosporin A therapy and on the next day started IFNα-2a therapy with 6 million units/day subcutaneously. Six days later the visual acuity rose to 20/40 in the right eye and 20/300 in the left eye. Funduscopy revealed a decrease of vitreous infiltration, macular oedema, and retinal haemorrhages in both eyes; the optic disc oedema in the left eye and the optic disc neovascularisation in the right eye regressed. Seventeen days after initiating IFNα-2a treatment the ophthalmological examination disclosed a stable visual acuity in the right eye and an increased visual acuity of 20/200 in the left eye because of irreversible macular defects. The retinal vasculitis had improved in both eyes; additionally, optic disc neovascularisation in the right eye had completely disappeared. Another 5 weeks later there was further improvement with a visual acuity of 20/25 in the right eye (Fig 2) and 20/100 in the left eye.
When tapering down the interferon therapy to 3 million units IFNα-2a per day the patient had one relapse in the end of October 1999. Since then he has had no recurrences and a stable visual acuity of 20/25 in the right eye and 20/100 in the left eye since November 1999 with a dosage of 3 million units/6 million units interferon alfa every other day.
We have already shown the efficacy of IFNα-2a in posterior uveitis and especially in retinal vasculitis with reopening of occluded vessels,1 but this case underlines the antiangiogenic effects of this cytokine in retinal neovascularisation.
It is known that IFNα-2a, a genetically engineered copy of one of the naturally occurring human interferons, has antiviral, immunoregulatory, and antineoproliferative properties and has been used clinically for many years in the treatment of viral hepatitis, solid tumours,2 and haematological malignancies. More recently it has been used successfully to treat haemangiomas of infancy and childhood, where an antiangiogenic effect has been demonstrated.3
Up to now, this antiangiogenic effect was examined in ophthalmology for treatment of choroidal neovascularisation in age related macular degeneration4 and for treatment of diabetic proliferative retinopathy,5 but it failed to show an effect in controlled clinical trials.
This case report hints at the possibility of treatment by IFNα-2a and, eventually, prevention of retinal neovascularisations due to ocular inflammation.
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