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Vogt–Koyanagi–Harada syndrome: importance of early treatment

We present previously healthy 26-year-old female patient that was examined in neurological emergency room with decreased visual acuity in both eyes associated with frontal headache, nausea and pressure in the ears. Due to these symptoms she was hospitalized in our Department. She was examined by ophtalmologist and her best-corrected visual acuity (BCVA) in the right eye was 0.3 on the Snellen charts and finger counting in the left eye. Anterior segments were normal, without inflammatory signs. Fundus examination revealed serous retinal detachment of posterior pole and optic disc hyperemia in both eyes. (Fig. 1a, b). Fundus fluorescein angiography (FA) showed angiographic features typical of VKH disease in both eyes: irregular patchy fluorescence of the choroidal circulation, multifocal pinpoint leakage in the early stage, pooling and dye leakage in the subretinal space in the late stage of FA. (Fig. 1c, d). Optical coherence tomography (OCT) showed large retinal detachment with subretinal septa creating pockets of fluid in the outer retina (Fig. 1e, f). The visual evoked potentials (VEP) found a reduction in the amplitude of P135 and the impairment in conduction of axons within the visual pathway. Extended laboratory tests of serum and cerebrospinal fluid were performed followed by radiological examination. Serology for syphilis, HIV and Borrelia burgdorferi was negative. Aquaporin antibodies were also negative. Chest radiograph and quantiferon test excluded tuberculosis. Magnetic resonance imaging (MRI) of the brain and orbits was normal. Human leukocyte antigen analysis (HLA) found positive HLA DQ3 and HLA B 51. Early in the course of disease we started therapy with intravenous methylprednisolone, 1000 mg for 5 days followed by high dose oral steroids tapered very slowly. Four weeks after the beginning of oral steroid therapy, oral cyclosporine A 125 mg twice daily was introduced with a regular measurement of therapeutic drug concentration in the serum. Five months after the initial clinical presentation visual function and retinal status were improved. The right eye BCVA was 0.8, and the left eye 0.8 on the Snellen charts. There is a noticeable and almost complete resorption of the subretinal fluid and retinal reattachment confirmed by optical coherence tomography (Fig. 1g, h).

Vogt–Koyanagi–Harada (VKH) disease ; prompt therapy

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