CASE REPORT

An 18 year old man with BD was admitted in January 2001. He had been diagnosed with BD four years earlier in view of his presentation of recurrent oral and genital aphthous ulcers, polyarthritis, erythema nodosum, and superficial thrombophlebitis. The onset of the ocular disease was in 1999, when the patient was treated with steroids and cyclosporin for bilateral posterior uveitis. In the course of cyclosporin treatment he had several attacks of uveitis in both the eyes. Cyclophosphamide was introduced without a satisfactory control of disease symptoms and of the ocular manifestations.

A new relapse of severe neuroretinitis occurred in October 2000. He was treated with intravenous methylprednisolone, followed by oral prednisone (50 mg/day), topical steroids, and mydriatic agents. Tapering of the prednisone dose resulted in November in a new acute attack of neuroretinitis in the left eye. Intravenous methylprednisolone was reintroduced, followed by 75 mg of oral prednisone and by local peribulbar injection of methylprednisolone every 15 days. Recovery was slow and less evident and, the visual acuity being 20/30, optic disc oedema and retinal vasculitis were still present. The patient received prednisone maintenance treatment (15 mg /day) for approximately four weeks before receiving an infliximab infusion.

At admission, fluorescein angiography (fig 1A) showed a hyperfluorescent optic disc in both eyes, and diffuse irregular mottled retinal hyperfluorescence and haemorrhagic hypofluorescence in the left eye. Oral and genital ulcerations were present together with erythema nodosum, thrombophlebitis, and arthritis. An infusion protocol was designed and approved by the Department of Internal Medicine Institutional Board and informed consent for treatment was obtained from the patient.

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Figure 1

(A) Fluorescein angiography obtained at admission, showing a hyperfluorescent optic disc in both eyes, diffuse irregular mottled retinal hyperfluorescence, and haemorrhagic hypofluorescence in the left eye. (B) Fluorescein angiography obtained before the third infusion, showing a normal optic disc aspect, improvement of macular oedema in the right eye and still in the mottled aspect of retinal capillary filling.

The patients was infused with infliximab, 5 mg/kg, by a two hour infusion, at weeks 0, 2, 4, and 8, and the patient observed for a further two hours without adverse effects. An improvement in symptoms was noticed within 24 hours after receiving the first infusion. At the time of the second infusion he had a complete remission of all signs and symptoms. A new fluorescein angiography was performed just before the third infusion. At that time there was a normal optic disc aspect, improvement of macular oedema in the right eye and still in the mottled aspect of retinal capillary filling (fig 1B). Before the first infusion the erythrocyte sedimentation rate was 35 mm/1st h and the C reactive protein level was 34 mg/l. They decreased to 22 mm/1st h and 6 mg/l (normal <10 mg/l), respectively, by week 2 and remained within the normal range for the duration of the study.

DISCUSSION

This is the first report, to our knowledge, of the treatment of ocular BD with anticytokine specific treatment. Treatment with infliximab led, in our patient, to a complete remission of all disease manifestations and there was no recurrence after steroid tapering.

Three interesting points can be made. Firstly, the drug had a profound effect on ocular BD as well as on the other manifestations of disease. This effect on global diseases seems to be remarkable, as standard treatments had failed in our patient. Secondly, the onset of improvement was fast. Thirdly, when a loading dose regimen of four infusions (weeks 0, 2, 6, and 8) was used, remission continued for up to eight weeks. Further confirmation of the beneficial effects of TNFα blockade in randomised, controlled, double blind studies is necessary.