Sir,

There are rare reports showing AION in Behcet's disease. Here, we report a case of AION in Behcet's disease with cilioretinal artery occlusion that was effectively treated by early oral corticosteroid treatment.

Case report

A 31-year-old man, diagnosed as having Behcet's disease 4 years earlier, presented with sudden reduced visual acuity in his right eye. He had been treated with oral prednisolone intermittently. At the first visit, his visual acuity was 20/80(OD) and 20/20(OS). He had recurrent oral aphthae and erythema nodosum on his lower legs. Upon fundus examination, there was a wedge-shaped ischaemic area with macular oedema on the papillomacular region (Figure 1a, left). Fluorescein angiography (FAG) identified an ischaemic area supplied by the cilioretinal artery and a focal area blocked by splinter haemorrhage (black arrow in Figure 1b, left). There was a caeco-central visual field defect in the right eye (Figure 1c, left). C-reactive protein was markedly elevated (22.43 mg/l). The patient was diagnosed with AION in Behcet's disease based on ocular involvement, oral aphthae, and skin lesion. He was prescribed oral prednisolone (40 mg/day). After 3 days, macular oedema was completely resolved (Figure 1a, right). After 1 month, his visual acuity improved to 20/25. Follow-up FAG showed neither ischaemic lesions nor macular oedema (Figure 1b, right). The visual field defect was still present; however, it had markedly decreased in size (Figure 1c, right).

Figure 1
figure 1

Colour fundus photographs at the first visit (left) and at 3 days follow-up (right). A wedge-shaped ischaemic area on the papillomacular region and a splinter haemorrhage (black arrow, inset) at first visit (left) resolved after 3 days (right). Macular optical coherence tomography at the first visit (left) and 3 days follow-up (right), showing the complete resolution of initially detected macular oedema (a). Fluorescein angiography at the first visit (left) and 1 month follow-up (right), showing an ischaemic lesion (arrowhead) and a focal area blocked by splinter haemorrhage (black arrow) initially (left), which resolved after 1 month (right) (b). Visual field tests at the first visit (left) and at 1 month follow-up, showing the visual field defect initially detected and its subsequent decrease in size (right) (c).

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Comment

In 1976, Scouras and Koutroumanos1 were the first to describe AION in Behcet's disease. AION is an infarction of the optic nerve caused by inadequate perfusion through the posterior ciliary arteries. Although the pathogenesis of AION in Behcet's disease has not yet been established, the periarteritis is known to be related to AION.2 Our case resembles arteritic AION, because of the vasculitis associated with cilioretinal artery occlusion. It was reported that a third of the patients with optic neuropathy in Behcet's disease lost their sight.3 Unlike the poor prognostic features of optic neuropathy in Behcet's disease, ischaemic optic neuritis was treated well with oral corticosteroids in rare reported cases.4, 5 Our patient also showed a relatively good corticosteroid treatment response and maintained a good visual acuity during 6 months of follow-up. Although our case cannot explain the overall clinical outcome of AION in Behcet's disease, this rare AION with cilioretinal artery occlusion was effectively treated by early oral corticosteroid treatment.