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Central Retinal Vein Occlusion in Behçet’s Disease

1Klais C., 2Grupchev I., 2Donaldson M.,
1The University of Auckland, Department of Ophthalmology (Auckland)
2Department of Ophthalmology, The University of Auckland, Private Bag 92019 (Auckland)

Purpose: Behçet’s disease is a rare autoimmune disease of unknown origin that mainly affects young men. Bilateral acute iridozyklitis is the typical ophthalmological feature.
Case report: A 45-year old Korean female presented with sudden unilateral loss of vision in December 2001. The right visual acuity was reduced to counting fingers, the left 1,0. Right biomicroscopy revealed some cells and flare in the anterior chamber as well as massive cell infiltration in the vitreous. The ophthalmoscopic appearance was characteristic for central retinal vein occlusion (CRVO) with papilloedema, hemorrhages, dilatation of veins, cotton wool spots and macular oedema. The vessels along the superior arcades showed extensive exudations. Examination of the fellow eye was normal. The patient reported having had oral and genital ulcerations for the past three years. In addition she had a skin rash last year, suggestive of erythema nodosum. All serological results were normal. The search for HLA-B5 and HLA-Bw-51 was negative. The medical consultant agreed with the diagnosis of Behçet’s disease despite of the absence of ulcers at the time of consultation. Intravenous therapy with methylprednisolone 1g/d for three days was initiated and then switched to high dose oral steroid. This dosage was slowly reduced to a maintenance dose (7,5 mg/d). Intraocular inflammation responded well to systemic treatment. We observed a regression of exudations, dilatation of veins and papilloedema. Macular oedema remained but visual acuity improved to 0,16. Due to persisting ischaemic changes the patient was closely observed. Until March 2002 no neovascularisations were detected.
Conclusions: In patients with CRVO systemic autoimmune disease should be considered especially in young patients in order to be able to initiate immunosuppressive treatment as soon as possible.